1. Not all arthritis is created equalIn my years in practice as a rheumatologist, I have noted that among patients and the medical community alike, there are some major misconceptions about arthritis.
The most common misconception may have come from the slogan “Arthritis: there is no cure.” Many equate the slogan to “Arthritis: there is no treatment.” Time and again, I have seen patients who have been told, “It’s arthritis and nothing can be done, so go home and live with it.”
The most dangerous misconception is that arthritis is not a serious disease. In fact, arthritis is the number one cause of disability in North America and affects people of all ages: it is not only a disease of the old.
It is also not true that all arthritis is the same: there are approximately 100 forms of arthritis. The most common form is osteoarthritis, which affects approximately 55 percent of us by age 65. Rheumatoid arthritis is less common, but does affect approximately 1 percent of the population; most of these people begin to be affected between the ages of 30 and 50.
What Is Arthritis?
Arthritis is derived from the Greek arthron (joint) and itis (inflammation). Therefore, arthritis by this definition would mean inflammation within a joint.
A joint is the structure that connects two bones. The joint is held together by ligaments and tendons and a capsule. The ends of the bones are covered with cartilage and the capsule is lined by a synovial membrane (see illustration below).
Cartilage is the white glistening stuff you have probably seen at the end of a chicken bone. It allows bones to glide smoothly over each other, with synovial fluid acting as a lubricant, and also absorbs the forces that impact on the joints. Cartilage is made up of collagen (protein fibres), water, chondrocyctes (the cartilage cells), and proteoglycans. Normal cartilage is essential to a well-functioning joint.
In arthritis, the cartilage may have become damaged. It may have thinned and eventually disappeared, leading to bone rubbing on bone. The damage to cartilage can occur in different ways, and this is why we have different forms of arthritis. The two major types of arthritis are degenerative (wear and tear) and inflammatory.
Degenerative arthritis is known as osteoarthritis. In this form of arthritis, the primary problem occurs in the cartilage. The cartilage may be damaged by a previous injury to the joint, or by a chronic stress on the joint (as in osteoarthritis in the knees of obese females). The cartilage itself may be abnormal because of a genetic defect that can lead to premature osteoarthritis in whole families. Degenerative arthritis used to be referred to as “non-inflammatory arthritis,” however, researchers have become increasingly aware that inflammation is an important factor in the progression of osteoarthritis.
In inflammatory arthritis, the inflammation in the joint causes the damage. The signs of inflammation are redness, heat, swelling, and pain. In the joint, this leads to loss of function. An example of an inflammatory arthritis is rheumatoid arthritis, in which the immune system is overactive, leading to inflammation mainly (but not always) in the synovium and the joint fluid. The inflammation itself causes pain and a poorly functioning joint, but ultimately, the cartilage is damaged and the joint is finished. Rheumatologists want to treat the inflammation before this happens.
Other conditions such as bursitis or tendonitis are not arthritis. They do not occur in the joint. They are referred to as soft tissue rheumatism, and are discussed in Chapter 2, “Not All Musculoskeletal Pain Is Arthritis.”
Osteoarthritis
Osteoarthritis is a common disease characterized by pain in the joint, stiffness, and loss of movement. It is also referred to as degenerative joint disease and osteoarthrosis. It is a slowly evolving disease that is part of the aging process. Autopsy studies have shown that by age 40, 90 percent of all persons have changes in the weight bearing joints (knees, hips) although, of course, all of these people did not have symptoms. By age 65, approximately 60 percent of us will have symptoms of osteoarthritis.
Not only is osteoarthritis common, but it has been around for a long time: dinosaur bones and Egyptian mummies all show evidence of osteoarthritis. Yet we still understand very little about this disease and specific treatment has eluded us.
Osteoarthritis is classified as a non-inflammatory arthritis. This implies that there is no inflammation, but recent research shows that this is not true. The factors that cause the disease (which starts in the cartilage) are not completely known. It is known that early on, although there is no inflammation yet, the cartilage starts to wear away and fragment, and the bone under the cartilage thickens. Loose pieces of cartilage cause an immune response and inflammation, which is known to be responsible for some of the progression of the disease. As the disease evolves, the cartilage thins and disappears. As well, new bone is formed at the joint edges (this is referred to as an osteophyte) and the bone under the cartilage continues to thicken.
Dr. Stein discusses in detail some of the causes of osteoarthritis in Appendix I, “Heredity and Environment,” but we will summarize some of the causes here:
• Trauma may lead to osteoarthritis (whether this is sudden, severe trauma, or a repetitive strain such as seen in obese females and osteoarthritis of the knees).
• The tendency to develop osteoarthritis can also be passed from parent to child in the genes, as we see with congenital hip dysplasia or erosive osteoarthritis of the hands (which seems to be passed from mother to daughter most commonly).
Classification of Osteoarthritis
Generally, osteoarthritis is divided into primary and secondary forms: primary osteoarthritis has no underlying cause; secondary osteoarthritis may be a result of trauma, previous inflammatory diseases such as rheumatoid arthritis, or crystal induced diseases.
Primary osteoarthritis is further broken down into the subsets of generalized osteoarthritis, erosive osteoarthritis, diffuse idiopathic skeletal hyperostosis, and chondromalacia patellae. These are discussed below.
Generalized osteoarthritis
Generalized osteoarthritis refers to osteoarthritis that affects the DIP (distal interphalangeal), PIP (proximal interphalangeal), and CMC (carpometacarpal) joints of the hands, but may also involve the neck, low back, hips, knees, and big toes (see illustration on page 5). There is usually a period where the joints are inflamed. Generalized osteoarthritis occurs primarily in middle-aged women.
Erosive osteoarthritis
This particular variant, which is usually inherited, involves the DIP and PIP joints and is associated with swollen, painful finger joints. The X-rays show erosions in the bone.
Diffuse idiopathic skeletal hyperostosis (DISH, or Forestier’s disease)
This is arthritis in the spine, in which huge flowing bone spurs (osteophytes) can be seen on X-ray between the vertebrae of the thoracic spine. Large bony spurs can also occur elsewhere, such as on the heels. Stiffness of the spine is the predominant symptom and pain may be surprisingly minimal.
Chondromalacia patellae
This is a condition seen in teenage girls, and more commonly in those who have had repeated trauma to the knee. They have knee pain that is made worse with squatting, kneeling, and going down stairs. What has happened is that the cartilage behind the kneecap has softened and split into fibres, and the kneecap has been allowed too much sideways movement because of looseness in its supporting muscles and tendons. What is needed is an exercise program to strengthen the muscles, after which the cartilage can go back to normal.
Symptoms
The joints that are commonly affected by osteoarthritis include the DIP, PIP, and CMC joints of the hands, the neck, lower back, hips, knees, and the big toes (or first metatarsal phalangeal joint). (See again the illustration on page 5.) If other joints are involved, it is usually as a result of trauma to that joint or an inflammatory arthritis (which will be discussed on page 15 of this chapter).
Pain
Pain is a predominant feature of osteoarthritis. Early in the disease, the pain accompanies movement, is aggravated by prolonged activity, and is made better with rest. When the condition is more severe, there is pain at rest and it will also awaken you at night.
The pain in osteoarthritis is not coming from the cartilage, which does not have nerve endings. The pain may be from the joint capsule, or from tendons around the joint that are being stretched because of the swelling or deformity of the joint. The pain may also be caused by stretching of the lining of the bone (periosteum) at the sites of new bone growth. If the bone spurs protrude into the space where the nerves travel (as in the neck or back) then the pain is from the nerve irritation or compression. Such a pain would travel down your arm or leg and would be a burning type of pain; there may be weakness or loss of feeling in the affected limb.
Stiffness
People with osteoarthritis often experience morning stiffness that usually lasts for less than 30 minutes.
Swelling
Once the cartilage has been damaged, bits of it actually cause an immune response and associated swelling, heat, redness, and pain (inflammation). Another cause of an acutely inflamed joint in osteoarthritis is the presence of calcium crystals in the joint space. Sometimes, the joint may appear to be swollen but it is actually enlarged as a result of the new bone growth. This is referred to as bony enlargement.
Crepitus
Crepitus is the grinding and creaking of the joint as it is being moved. This is common in osteoarthritis.
Deformity
The bony enlargement of the DIP joint of the finger is called a Heberden’s node. The enlargement of the PIP joint is called a Bouchard’s node. These can begin as a swelling like a cyst filled with jelly-like material. At times they become inflamed and painful. Once the inflammation has settled, they stop hurting and become hard and bony.
At the base of the thumb is the CMC joint, which can be involved with osteoarthritis. The joint can be very painful, like a boil, and becomes enlarged and bony. Often physicians refer to this as squaring of the joint.
There are two common deformities of the knees. The first, genu valgus or knock knees, occurs with loss of the lateral joint space of the knee and is more common in women. The second, genu varus or bow legs, is due to loss of the medial joint space of the knee and is more common in men.
The other common deformity of osteoarthritis is a hallux valgus deformity of the big toe: this is your common bunion.
Investigations
There are no specific laboratory tests for osteoarthritis: the diagnosis is based on the clinical findings and X-rays. This disease stays in the joints and the tests for inflammation (such as the ESR or the CRP) are usually normal.
The typical changes we see on X-ray include loss of cartilage, thickening of the bone under the cartilage, and bony spurs. You do not usually need more sophisticated X-rays, such as a CAT scan (computerized axial tomography) or MRI (magnetic resonance imaging). The MRI does show the cartilage changes better, however, and so would probably be used in clinical studies where it would be important to determine whether new therapies have an effect on the cartilage.
Management
The management of osteoarthritis includes treating the pain, maintaining the range of motion of the joints, and preventing disability. To do this, patients use medications, exercise, supportive devices, modification of daily activities, and surgery. To date, the most significant improvement in the management of osteoarthritis has been the replacement surgery for the knee and hip.
Medications
Medications are discussed much more fully and in more detail in Chapter 7, “Medications.” This section is intended to be an overview of medications available for treating osteoarthritis in particular.
No medication is currently available that repairs the cartilage, although considerable research is being done into the development of what are termed the “disease modifying osteoarthritic drugs.”
There is no harm in trying acetaminophen first. It should be taken on a regular basis, up to 650 mg (or two regular-strength pills) four times a day. If this is not working, then an NSAID (non-steroidal anti-inflammatory drug) can be tried, provided there is not a contraindication to its use. If you have had a previous bleed from a stomach ulcer, then you should be on something to protect your stomach (such as misoprostil or a proton pump inhibitor) or you could take a COX-2 selective drug, which is easier on the stomach. If you have high blood pressure, kidney problems, or congestive heart failure, then check with your physician before using an NSAID.
There are several topical agents used in osteoarthritis for pain control. Most of these contain capsaicin, which is derived from cayenne pepper. These products are rubbed over the skin three to four times a day, and may provide moderate relief of pain. There are also topical non-steroidal preparations, but the difficulty with these is that we don’t know how much is absorbed, and they have the potential for the same side effects as NSAIDs that you take by mouth.
There are several products that are labelled as viscosupplements (including Synvisc and Orthovisc). These are derivatives of hyaluronic acid, which is a naturally occurring part of the joint fluid, and they are given to supplement or add to what the body already makes. These drugs are given as a weekly joint injection (usually into the knee) for three weeks. They help with pain and improve mobility in 50–60 percent of cases. This effect lasts approximately six months to one year. Viscosupplementation works best in patients who have mild to moderate osteoarthritis of the knee.
Low doses of amitriptyline or other tricyclic antidepressant medications are very useful for pain control. They are used at night in small doses and they help you with sleep, have a direct effect on pain, and help relax your muscles.
Narcotics are occasionally recommended for the control of chronic pain and are sometimes necessary to control the pain of osteoarthritis.
As mentioned, disease modifying osteoarthritis drugs are not available yet, but glucosamine and chondroitin sulfate have been used to treat osteoarthritis. These are available in pharmacies and natural food and supplement stores. These products have been studied in Europe more than in North America, but the results to date suggest that both have an effect on pain, although the better designed studies showed them to have less of a benefit than placebo. Both are well tolerated; some people reported stomach upset, but this was mild. There may be an increase in blood sugar levels in diabetics on glucosamine, so this should be monitored. These products are not regulated, and it is therefore advisable to buy from a reputable manufacturer to ensure that you have purchased the advertised compound. There is a website (www.drtheo.com) that reports on the independent laboratory analysis of products from the nutriceutical industry, and you might do well to check the website to get unbiased information about a product that you’re thinking about taking.
Corticosteroids are only used to treat osteoarthritis by injection into the joint, which provides short-term relief (usually in the range of four weeks).
Tetracycline and minocycline are antibiotics that may actually help prevent cartilage loss in osteoarthritis, and clinical studies are now underway to investigate this.
Therapies
A physiotherapist can help to settle an acute flare of osteoarthritis, and can also provide an appropriate exercise regime, which is essential for patients with osteoarthritis. Acupuncture and massage are often very beneficial in the control of arthritis pain.
This next section will cover specific therapies that rheumatologists have found helpful for the management of osteoarthritis in specific joints.
Osteoarthritis of the hands usually affects the DIP, PIP, and CMC joints. As these joints can frequently be inflamed, it is useful to use an NSAID when the joint is swollen, red, and tender. Some people find that they need these drugs only periodically, while others need them on a regular basis. If you are using them as an anti-inflammatory, then they need to be taken for at least two weeks on a regular basis to have their full effect. If one or two joints are particularly inflamed, then an injection of corticosteroids into the joint is helpful (particularly for the CMC joint).
Hot wax therapy makes the joints feel good. If this is not possible, you might get a similar effect by rubbing mineral oil into your hands, putting on tight rubber gloves, and putting your gloved hands into hot water.
It is important to exercise your hands regularly so that you prevent loss of range of motion.
Occasionally, surgery is necessary if you are experiencing pain that is not otherwise controlled or if there is progressive deformity. The CMC joint can be replaced or fused; the surgery works well. The DIP joint can be fused. The PIP joint can be replaced or fused.
Osteoarthritis in the cervical spine occurs in the small joints and is usually associated with degeneration of the discs and bone spur formation. Arthritis of the cervical spine leads to a stiff and painful neck and limited range of movement. The muscles may also be in spasm, and patients tend to sleep poorly.
Management first involves avoiding activities that might aggravate the pain. For example, if your job requires you to talk on the phone a long time, you should use a headset. If you are working on a computer, make sure that it is at a proper height and that you have a comfortable chair. This is not to say that you should cease all activity, however, because exercise is important for maintaining range of movement and strength. Even a few simple stretches in the shower are helpful.
It is important to get a good sleep. Cervical pillows are helpful, and the water-filled pillows seem to work best for most people. Sometimes a low dose of a tricyclic antidepressant is very useful as it relieves the pain, gets you to sleep, and also relieves the muscle spasm. NSAIDs are useful to try, and some people find it helpful to take regular Tylenol or Tylenol with codeine at bedtime.
A soft collar is helpful if you are having a lot of pain. It should not be worn for more than a few hours a day, however, as wearing it too long leads to muscle weakness and worsening of the problem.
Surgery is an option if the nerve root is irritated or compressed to the point that pain is running down the arm and there is numbness or loss of strength in the hand. These types of symptoms are referred to as radiculopathy. The symptoms will sometimes settle on their own, however if they are persistent or severe, then surgery may be needed.
Osteoarthritis in the lumbosacral spine occurs at the back of the spine at the facet joints and the discs. The treatment includes NSAIDs, painkillers (narcotics), physiotherapy, exercise, and supportive devices.
In the condition called spinal stenosis, the spinal cord and nerves coming from the cord are being crowded or pinched by the bone spurs or protruding discs. The pain occurs when walking, is improved with leaning forward, and is relieved with a few minutes’ rest. This condition, if severe, requires surgery.
Patients with pain from osteoarthritis in the facet joints often experience pain in the buttock region. Many refer to this as hip pain, although it is not from the hip but from the lower back. This pain may radiate down the back of the leg to just above the knee, and is made worse by arching the back. The best treatment is an exercise program to strengthen the muscles. Physiotherapy can be helpful for some to settle the pain, but an exercise program is needed to prevent the pain from recurring. A girdle or a lumbosacral corset can be helpful if you have to stand for any length of time. Do not use the corset too much, however, as this can lead to muscle weakness.
Lower back pain that is originating with the discs is usually across the mid-lower back. If a nerve is being pinched, then the pain will radiate down the whole leg and there may be numbness or loss of strength in the leg. This is, again, a radiculopathy. The treatment includes an exercise program and pain medication. If the pain is persistent and severe, surgery may be needed.
The symptoms of osteoarthritis of the hip are pain, stiffness, and loss of range of movement. Hip pain is usually felt in the groin and down the thigh, and may sometimes be felt in the knee (this is a referred pain pattern for the hip). One of the first symptoms may be difficulty in putting on your socks or pantyhose.
Continuing to walk is important, as are range-of-motion exercises. Impact sports (such as running, basketball, or squash) should be avoided. If you are experiencing a lot of pain on walking, then it is important to use a cane. (If your left hip is involved, you carry the cane in your right hand and put your left foot forward as the cane moves forward on the right side.) This will take 40 percent of your weight off the bad hip, which can make a huge difference.
The use of NSAIDs and acetaminophen can be helpful. Once the pain and loss of movement are significantly interfering with your lifestyle and/or you are waking at night with pain, you might consider a hip replacement. Because the artificial hips do not last forever and each repeated surgery is more difficult, surgeons ask their younger patients to delay the surgery as long as possible.
Osteoarthritis of the knees causes pain, stiffness, and loss of mobility. An exercise program is essential to maintain strength and range of motion. If the knee pain occurs mainly with kneeling, squatting, or walking downstairs, then the pain may be caused by the kneecap scraping against the knee. This is best managed with physiotherapy and an exercise program for patellofemoral disease.
Using a cane can be very beneficial with knee osteoarthritis, as it removes 40 percent of your weight from the knee. A support bandage or brace may sometimes provide stability and comfort.
Swelling is often associated with osteoarthritis of the knees, and NSAIDs can be beneficial. If these do not settle the swelling, an injection of corticosteroids can help.
Patients with mild to moderate osteoarthritis and those for whom surgery is contraindicated may wish to try viscosupplementation (see Chapter 7, “Medications”) for some relief of the pain on walking.
Arthroscopic surgery to clean out the joint may temporarily help with the symptoms of osteoarthritis of the knee, and a high tibial osteotomy for osteoarthritis of the medial joint space can buy time before a knee replacement. If the above have failed, however, and you are experiencing significant pain, loss of function, and night pain, a knee replacement needs to be considered. (Please see Chapter 10, “Surgery.”)
Osteoarthritis of the first MTP joint (also known as a bunion joint) is a very common problem. The initial treatment is to buy shoes, with or without orthotics, that are comfortable and accommodate your foot. If the pain is interfering with activities, then surgery can be considered, but this surgery is not minor: recovery takes from six weeks to three months. (Again, please see Chapter 10, “Surgery.”)
Rheumatoid Arthritis
Rheumatoid arthritis is the most common form of chronic inflammatory arthritis. It affects 1 in 100 people, and roughly 300 000 Canadians have the disease. Three times as many females as males are affected, and it can occur at any age, although the most common age of onset is between 30 and 50 years.
Rheumatoid arthritis causes swelling, pain, and stiffness of the small joints of the hands, wrists, elbows, knees, and feet. It can, in fact, affect almost any joint, but usually spares the lower back and thoracic spine. Rheumatoid arthritis is also a systemic disorder, meaning that more is affected than the joints: the eyes, lungs, small blood vessels, and other organs can all be involved. People with rheumatoid arthritis experience fatigue, anemia, and a general feeling of being unwell. They are usually stiff in the mornings for more than an hour, and the more severe the arthritis, the longer the morning stiffness will last.
In autoimmune disorders in general, the immune system is revved up and attacks one’s own tissues, cells, or molecules. In the case of rheumatoid arthritis in particular, most of the action is at the joint, although other tissues are involved. Other examples of autoimmune diseases include diabetes, thyroid disease, systemic lupus, erythematosus, and Crohn’s disease.
This immune response in rheumatoid arthritis causes inflammation and thickening of the lining of the joint (the synovium), and causes fluid to move into the joint space (see the illustration of a rheumatoid joint below). This leads to a swollen, hot, painful joint. The immune cells release hormones called cytokines, which control the release of enzymes that damage the cartilage, bone, ligaments, and tendons. New therapies for rheumatoid arthritis inhibit the bad cytokines or promote the good ones. The cytokines that have become the targets of therapies are tumor necrosis factor alpha (TNFa) and interleukin-1 (Il-1).
Please see Appendix I, “Heredity and Environment,” for further information on the causes of rheumatoid arthritis.
Symptoms
Rheumatoid arthritis begins differently in different people. Some awaken in the morning with diffuse swelling in the hands, wrists, ankles, and feet.
Others have swelling in a joint for 48 hours and then the swelling disappears, but soon recurs in another joint. The arthritis migrates from joint to joint. This is called palindromic rheumatism, and approximately one-third of the time it settles into rheumatoid arthritis.
In elderly patients, the arthritis may start with severe stiffness and discomfort in the shoulders and hips, mimicking a condition called polymyalgia rheumatica. However, the development of swelling in the hands or feet makes rheumatoid arthritis a more likely diagnosis.
Some people will initially be affected only in one or two joints but with time, many other joints become involved. This is referred to as an additive pattern.
The general symptoms or signs of rheumatoid arthritis may be fatigue, morning stiffness lasting longer than an hour, and swollen, tender, and warm joints. The joint involvement is symmetrical: the same joints are involved on both sides of the body. It is more likely to affect the peripheral joints (i.e., the hands, wrists, ankles, and feet). Rheumatoid arthritis affects the PIP and the MCP joints in the hands but spares the DIP joints. It affects the MTP joints of the feet. (See illustration on page 19.)
The American College of Rheumatology has identified the following seven criteria for the diagnosis of rheumatoid arthritis:
- Morning stiffness: stiffness in and around the joints lasting at least one hour until maximal improvement
- Arthritis of three or more joints
- Arthritis of the hand joints: at least one area swollen in a wrist, PIP, or MCP joint
- Symmetric arthritis
- Rheumatoid nodules
- Serum rheumatoid factor
- Radiographic changes typical for rheumatoid arthritis
A patient must have four out of the seven to satisfy criteria, and the first four conditions in the list above must have been present for at least six weeks. A patient may not meet criteria but still have rheumatoid arthritis.
Investigations
Early diagnosis and treatment of rheumatoid arthritis is important because damage can occur within the first three months of disease. Once damage has occurred, it is permanent. You should therefore see your family physician if you have joint pain, swelling, and stiffness. If rheumatoid arthritis is suspected, the physician should order the following tests and refer you to a rheumatologist.
Rheumatoid factor test
Also called a latex fixation test, this blood test measures for an IgM antibody to IgG; this antbody is called the rheumatoid factor. Of patients with definite rheumatoid arthritis, 90 percent have a positive rheumatoid factor. It can usually be positive in patients with the rheumatoid factor, but can also be positive with Sjogren’s syndrome (see page 35 of this chapter), with age, and with other chronic diseases (but usually at low levels). The rheumatoid factor test is useful but not essential in making a diagnosis in a patient with joint pain, stiffness, and swelling. High levels of the rheumatoid factor predict a more severe disease.
CBC
The CBC, or complete blood count, is a routine test for many conditions. The abnormalities that might be seen in rheumatoid arthritis are as follows:
- Anemia (low hemoglobin, low numbers of red blood cells) may be noticed. In active disease, patients may have what is called anemia of chronic disease.
- The platelet count may be increased if there is a lot of inflammation. Platelets help with blood clotting, and an increased count is usually not dangerous. In Felty’s syndrome, the platelet count can be low. (See the discussion of Felty’s syndrome on page 22, in the section on complications of rheumatoid arthritis.)
- The white blood cell count (WBC) is generally normal in rheumatoid arthritis. It can be low from medications or Felty’s syndrome, or with lupus. (See the discussion of lupus on page 29 in the section on connective tissue diseases.)
ESR
ESR, sed. rate, erythrocyte sedimentation rate: these are all different terms for the same thing. The ESR is an indirect measure of the amount of inflammation. The higher the level, the more inflammation there is.
CRP
The CRP, or C-reactive protein test, is another measure of inflammation. As with the ESR, the CRP test may be one of several things that would help your doctor decide if treatment is working.
ANA
The ANA, or antinuclear antibody test, is more commonly positive in lupus. Approximately 10 percent of patients with rheumatoid arthritis will have a positive ANA.
X-rays or radiographs
These are usually normal in early disease. The X-ray only shows changes when damage has occurred. In rheumatoid arthritis, changes occur first in the feet and hands.
Other Disease Manifestations and Complications of Rheumatoid Arthritis
Rheumatoid arthritis is a systemic disease, meaning that it affects more than the joints. Its most common effect on the body at large is fatigue, which is worse when the disease is more active and can be more bothersome than the pain or swelling. Controlling the underlying disease will improve the fatigue.
Nodules
Rheumatoid nodules are rubbery, firm, rounded lumps found under the skin in approximately 20 percent of patients who have rheumatoid factor positive disease. They are sometimes stuck to the bone and at other times are mobile, but they usually occur in places where you put pressure, such as where you lean on your elbows. Rheumatoid nodules can also occur in the lungs, and are not harmful.
There is no specific treatment for the nodules. If the disease gets better with treatment, they will sometimes recede. If they are surgically removed, they will return. Methotrexate can make them worse, and can cause more small nodules to appear on the fingers.
Eye effects
The most common effect on the eyes is that they become dry. Dryness of the eye is treated with lubricant drops or gel. The combination of dry eyes and dry mouth is referred to as sicca syndrome.
Inflammation of the sclera (the white covering of the eyeball) is called episcleritis. It can occur from the eye dryness or as a result of inflammation in the small blood vessels of the sclera. If the inflammation goes deeper, it is referred to as scleritis. If it is severe and left untreated, the eyeball can perforate. If you have a red and/or painful eye, you should see your doctor.
Lung effects
Lung involvement occurs in up to 28 percent of people with rheumatoid arthritis. The most common effect—thickening of the lung’s lining (the pleura)—does not usually cause any symptoms.
Sometimes people will develop pleuritis, in which the lining of the lung becomes inflamed (causing pain when breathing in) and the pleural space may become full of a fluid (pleural effusion). This sometimes will improve on its own, or may require treatment with a drug such as prednisone.
The lungs may become scarred as a result of chronic inflammation. This is called interstitial fibrosis, and is associated with more severe rheumatoid disease.
An extremely rare but very serious complication is called bronchiolitis obliterans. It is uncertain if this is due to the disease itself or if it is a complication of drug therapy (specifically gold or penicillamine). I have only seen one case in 12 years.
Felty’s syndrome
In this syndrome seen in rheumatoid arthritis, patients have an enlarged spleen and a low white blood cell count. They may also have a low platelet count, and bacterial infections are more common. It usually improves with DMARDs (disease modifying anti-rheumatic drugs) but sometimes the spleen has to be surgically removed.
Vasculitis
In rheumatoid vasculitis, inflammation in the small blood vessels causes them to become damaged and plugged off, cutting off the blood supply to the tissue that the vessel was supplying. Resultant damage can include leg ulcers, nerve damage that usually affects sensation in the arms or legs, loss of strength and ability to move, and gangrene in the feet or hands.
This condition is seen in severe rheumatoid arthritis in people with a high rheumatoid factor, and is rare. The treatment is high doses of corticosteroids and immunosuppressant drugs.
Ruptured popliteal cyst
This can occur with any condition that causes the knee to swell. The fluid in the swollen knee leaks back behind the knee through a one-way valve into a pouch (bursa). Since the fluid gets in but can’t get out, the bursa or cyst gets bigger and bigger until it bursts. This causes the lower leg to become swollen, very painful, red, and hot.
The management is first to make sure that the problem is not a blood clot. This is done using a Doppler machine (a type of ultrasound), which looks at blood flow. If it is not a blood clot, then the management is to keep the leg elevated and to put ice on it. Analgesia is given for pain control, and narcotics may be required. Usually a shot of corticosteroids in the knee helps.
Neck effects
Because rheumatoid arthritis also affects ligaments and tendons, it can damage many of the supportive tissues in the neck, leading to unstable movement of the neck. Sometimes your rheumatologist or surgeon may ask for special X-rays of the neck to be sure there is not too much unstable movement, especially when bending the neck. This is an important complication because if it is not detected, it can lead to damage of the spinal cord. If it is severe, then treatment is to perform surgery in which the neck is fused.
Chronic deformities
The chronic deformities of rheumatoid arthritis include, in the hand, ulnar drift of the MCP joints and Boutonniere’s or swan neck deformity of the fingers (see the illustrations on page 25). The wrist may rotate or drift towards the thumb. The elbow can become permanently bent. The shoulders usually are not deformed but they lose their range of motion. In the lower limbs, the legs may become bent at the knees and hips, and the ankles can roll inward. The big toe often drifts outward and the remaining toes bend upwards.
Control of the disease is the best way to prevent deformity. Splints may be used to prevent flexion contractions or bends in the joints. Wrist splints may be worn when working or at rest and may help prevent deformities of the wrist. Sterling silver ring splints can help prevent progression of the swan neck or Boutonniere’s deformity.
The deformities of the foot are best accommodated with orthotics and supportive shoes.
Prognosis
Rheumatoid arthritis is a chronic disease that causes erosions in the bone and cartilage of the joints, which can be detected by X-rays. The degree of erosion development predicts the severity of disability. At the time of diagnosis, up to 30 percent of people will already have developed erosions. Early aggressive treatment is necessary to prevent erosions and hence disability.
Rheumatoid arthritis affects individuals differently. Approximately 15 percent of patients will have a benign course and therefore have very little damage, if any. (In elderly patients, particularly if their disease begins with stiffness in the shoulders and hips, the disease is often benign.) For approximately 50–60 percent of patients, the disease runs a moderate course. However, 25–35 percent of patients will have serious disease that is difficult to manage with traditional therapies. Predictors of more severe disease include: a strongly positive rheumatoid factor; HLA DR4; young age of onset and female; extra-articular features (disease outside the joint, in lungs, eyes, etc.); uncontrolled polyarthritis; structural damage and deformity; and functional disability.
The ability to remain in the workforce is often determined by the physical demands in the workplace, the pace of work, and the amount of flexibility in the work schedule. Some studies have suggested that up to 50 percent of people with rheumatoid arthritis will be disabled and unable to work within five years of diagnosis.
A recent study suggests that the life expectancy of someone with rheumatoid arthritis is reduced by three years, on average. Those patients with more severe disease have a higher mortality rate.
Clearly, it is important to recognize the disease early and begin to treat it. For 20 years, we did not have any new therapies to offer patients. In the last five years, we have seen the introduction of the biological therapies and of a new drug called leflunomide, and have seen patients dramatically improve on these therapies when everything else has failed. Therefore, the future for people living with rheumatoid arthritis is better than ever before.
Management
The goal of therapy is to stop inflammation, control pain, prevent deformity, maintain function and, ultimately, prevent long-term disability. To achieve this requires a coordinated health care team that includes a physiotherapist, occupational therapist, nurse, family physician, orthopedic surgeon, and rheumatologist. Ideally, a patient with suspected or confirmed rheumatoid arthritis should see a rheumatologist within three months of the onset of the disease, since we know that irreversible damage can occur early. An early appointment is not possible in many parts of Canada, however, as there are not enough specialists.
Medications
Rheumatoid arthritis is usually treated with a combination of NSAIDs and DMARDs. Not all patients will respond to a particular medication. Often, a medication will work for a while and then become less effective. Most rheumatoid arthritis patients will, over the course of their disease, have been on a number of different NSAIDs and DMARDs. Some medications will have done nothing, some will have worked for a while, and some may have had to be stopped due to adverse effects.
The NSAIDs help with pain, swelling, and stiffness; however they alone do not control the disease process. Chapter 7, “Medications,” will deal with the benefits and side effects of all the medications.
As soon as the diagnosis of rheumatoid arthritis is made, a disease modifying antirheumatic drug (DMARD) should be started to control the disease process. Clinical studies have shown that starting any DMARD early in the course of the disease will decrease the rate of formation of erosions, whereas someone who begins a DMARD later in the course of the disease can never catch up. Some people believe there is a window of opportunity for treating RA early on before the inflammation process becomes too well established.
Methotrexate is the most commonly used DMARD to treat rheumatoid arthritis. All new drugs are compared to it in studies. It can be used alone or in combination with other DMARDs. Other DMARDs include sulfasalazine, hydroxychloroquine, minocycline, gold, penicillamine, azathioprine, and cyclosporine. Several studies have shown that the combination of methotrexate, sulfasalazine, and hydroxychloroquine is more effective than methotrexate alone or the combination of sulfasalazine and hydroxychoroquine, particularly in early disease. Leflunomide (Arava) is a newer DMARD with efficacy similar to methotrexate. It can be used alone or in combination with methotrexate. Please see Chapter 7 “Medications” for a further description of these drugs.
The biologic response modifiers have been introduced in the past five years. This group of drugs includes the TNF inhibitors such as adalimumab (Humira), etanercept (Enbrel), and infliximab (Remicade). They are more effective when combined with methotrexate and represent a major advance in the treatment of rheumatoid arthritis. These drugs benefit 60 to 70 percent of cases.
Corticosteroids are very useful drugs for patients with rheumatoid arthritis. They are very effective when injected into an inflamed joint, and if a patient has several joints that just will not settle, this approach is used. They are sometimes given as an injection into the muscle during an acute flare of disease. They are also at times given orally as a steady daily dose or in tapering doses, but we try to avoid using them over the long term because of their adverse effects. Remember that if you are on corticosteroids (prednisone) and you have been on them for awhile, do not stop them on your own. Consult your physician on how to wean yourself from them. You can become very ill if you stop them suddenly.
Self-help
It is always important to maintain a positive attitude, and to remember that you are in control. It is essential that you understand the goals of therapy, the potential adverse effects, and the expected outcomes; this applies to drugs, exercise, and surgery—really, to any therapeutic intervention. It is important to find a physician who is knowledgeable about rheumatoid arthritis, and whose advice you trust. At times you may receive conflicting information on how to proceed, and sometimes must trust your doctor’s clinical experience in having seen the same problem many times before.
You need to maintain as normal a lifestyle as possible, incorporating an appropriate exercise program and getting adequate rest, since fatigue is a huge problem for many patients. Exercise, splinting, pain control, lifestyle adjustments, and other forms of management will be dealt with in other chapters of the book.
So in summary, what can you do for yourself? Be informed, surround yourself with a heath care team whose opinions you trust, exercise, get enough sleep, eat well, don’t smoke, don’t waste your money on miracle cures, and stay in control.
The Connective Tissue Diseases
The connective tissue diseases are a group of autoimmune diseases that includes systemic lupus erythematosus (lupus), scleroderma, Sjogren’s syndrome, polymyositis and dermatomyositis, and vasculitis. These diseases have many clinical manifestations and a book could be written about each of them, but one of their common features is arthritis that does not usually damage the joints. We will discuss the conditions and management in a broad sense and refer you to more detailed resources if you wish to investigate these diseases further.
In all of these connective tissue diseases, the body’s immune system has an abnormal response to something foreign to the body (an antigen), and ends up producing antibodies against itself (autoantibodies). Some of the antibodies mediate the disease, but others do not.
Systemic Lupus Erythematosus
There are three classifications of lupus: discoid lupus, subacute cutaneous lupus erythematosus, and systemic lupus erythematosus.
Discoid lupus refers to a rash that occurs on sun-exposed areas. This rash is red, raised, and can cause scarring. It is uncommon for it to progress to systemic lupus erythematosus.
Subacute cutaneous lupus erythematosus refers to a recurring rash that is sun-sensitive. The rash can form rings or scaly patches. There can be arthritis, fatigue, and general malaise, but it is unusual to have serious organ involvement. This form of lupus is associated with the antinuclear autoantibody (ANA) directed against Ro and La. In a pregnant woman the Ro autoantibody can cross the placenta and cause the baby to have neonatal lupus. (Please see Chapter 4, “Pregnancy, Delivery, and Arthritis.”)
The arthritis associated with systemic lupus erythematosus is symmetrical and mainly involves the joints of the fingers, wrists, elbows, shoulders, knees, and feet. It can range from joint pains, with or without tenderness, to tender swollen joints. It can be mistaken for rheumatoid arthritis. The joints do not get damaged, but some patients develop swan neck deformities and ulnar drift.
Systemic lupus erythematosus (SLE) is more serious. It is associated with rashes (such as the butterfly rash on the face), reactions to sun exposure, hair loss, mouth sores, Raynaud’s phenomenon (where your fingers turn white and purple in the cold), inflammation around the lining of the lungs and heart, anemia, low white blood cell and platelet counts, and kidney disease with the potential for kidney failure. It may also affect the neurological system, causing seizures, depression, or numbness and tingling in the face or a limb.
The disease is characterized by cycles of getting better and then worse, but the outlook for people with lupus has improved because of better treatments. Although lupus can be a serious disease, 90 percent of patients do well. It affects women more commonly than men, and often starts in young women. There is an increased risk of cardiovascular disease in patients with longstanding disease, and it is therefore important to screen these people for other risk factors of cardiovascular disease.
The diagnosis is sometimes difficult to make in the early stages or with milder disease, but laboratory tests can help.
Investigations
The blood work abnormalities that can be found include a low white cell count, a low platelet count, and/or a low red cell count (anemia).
The autoantibodies that can be detected in lupus include antinuclear antibodies (ANA), such as those directed against DNA, Sm, Ro, and La.
The ANA is an antibody to the antigens in the nucleus of the cell. They are seen in many conditions but it would be most unusual to have systemic lupus reythematosus and negative antinuclear antibodies. The dsDNA and Sm are specific antibodies for systemic lupus reythematosus. The DNA is seen at high levels in active disease and is associated with kidney disease. The antibodies to Ro and La are associated with milder forms of lupus, arthritis, rash, and fatigue. These antibodies are seen in Sjogren’s syndrome and neonatal lupus. Please see Chapter 4, “Pregnancy, Delivery, and Arthritis.”
Antibodies to phospholipids are seen in a subgroup of lupus patients who have a tendency to form blood clots in arteries and veins and to have frequent miscarriages. There are three types of antiphospholipid antibodies: anticardiolipin, lupus anticoagulant, and a false positive test for syphilis.
Complement levels are used to follow disease activity: if they are low, it may mean that the disease is more active.
The analysis of the urine can be abnormal with protein and red cells and white cells. Such a result suggests that the kidneys are affected by lupus.
Treatment
The treatment of systemic lupus erythematosus depends upon which organs are involved and the severity of the disease. Although there is no cure, the disease can be controlled or it can spontaneously settle.
Hydroxychloroquine (Plaquenil) is helpful for the fatigue, arthritis, and rash associated with systemic lupus erythematosus, and probably prevents flares of the disease. Corticosteroids are used to treat fluid around the lungs or heart, kidney disease, more severe rash, arthritis, low blood counts, and neurological disease. Methotrexate is helpful in controlling moderately severe arthritis and rash. Azathioprine (Imuran) is used to treat moderately severe kidney disease. Cyclophosphamide is given as a large, slow injection of medication into a vein every four to twelve weeks to treat diffuse proliferative glomerulonephritis (a severe form of kidney inflammation) and neurological disease. Mycophenolate mofetil is a new immunosuppressant drug to treat kidney disease; at the present time, it is used when cyclophosphamide has failed or has not been tolerated.
Antiphospholipid Antibody Syndrome
This syndrome may occur in association with lupus or on its own. The main signs of this syndrome are blood clots in the veins and arteries, and recurrent miscarriages. The clots can be deep in the veins of the leg (causing phlebitis), lodged in the lung (known as a pulmonary embolism), or lodged in the brain (causing strokes). The syndrome can also be associated with low platelet counts, a rash called livedo reticularis, and heart murmurs. The syndrome in a pregnant woman can cause miscarriages and stillbirths, especially in the second third of the pregnancy.
The blood tests would reveal the anticardiolipin autoantibody, lupus anticoagulant (in a prolonged clotting test called a PTT), and a false positive result for syphilis.
The treatment is warfarin (Coumadin) to thin the blood and prevent further clots. If you have a positive blood test for this syndrome but have never had any symptoms, no treatment is required.
Scleroderma
Scleroderma is, fortunately, uncommon. It is estimated that a family physician would see one case in his or her entire career. It is three times more common in women, and usually occurs between ages 34 and 65. Scleroderma can be classified into three categories: cutaneous, limited, or diffuse.
In cutaneous scleroderma, only the skin is involved. There may be isolated patches of thickened skin (called morphea), or widespread skin thickening (called generalized morphea). Long strips of thickened skin are called linear scleroderma, and represent another type of cutaneous scleroderma; they soften with time.
Limited scleroderma was previously called CREST syndrome. Compared with diffuse scleroderma, it is milder and has a better prognosis. Its symptoms include thickening of the skin of the hands and feet (but not of the forearms or legs), and the skin on the face can sometimes be involved. In addition, there is Raynaud’s phenomenon, difficulty swallowing and heartburn (as a result of involvement of the esophagus), and the presence of tiny dilated blood vessels in the skin appearing as tiny red dots (telangectasia) and tiny calcium deposits under the skin (calcinosis). High blood pressure in the blood vessels of the lungs (pulmonary hypertension) is more common with this form of scleroderma.
Diffuse scleroderma (or progressive systemic sclerosis) is the most severe form of the disease. It causes widespread thickening of the skin that begins in the hands and the feet and spreads to the arms, legs, face, and trunk. The appearance of the face may change, as the skin thickening can cause pursing of the lips and loss of wrinkles. When there is rapid progression of the skin thickening in the early phases of the disease, it is a sign that the disease is serious and there is the potential for severe kidney problems. The leading cause of death with this disease used to be kidney failure: the blood pressure suddenly rose and the kidney function deteriorated. The development of the family of blood pressure pills (called ACE inhibitors) has dramatically improved the outcome of this complication. The leading cause of death from scleroderma now is progressive scarring of the lungs (interstitial fibrosis), which shows up as shortness of breath and a dry cough.
When scleroderma affects the esophagus, people experience heartburn, difficulty swallowing, and difficulty with food sticking in the throat. The speed of food passing through the bowel is slowed so that constipation and bowel obstruction can be a problem. The intestinal bacteria may become too numerous because of the slow bowel speed, and this will likely cause diarrhea that would be followed by malnutrition due to the inability to absorb all the nutrients.
Raynaud’s phenomenon, where the fingers and toes become white and then purple, results from the reduced blood flow to the extremities that is caused by narrowing and spasm of the blood vessels. It is brought on by cold temperatures and by anxiety. If severe, it can lead to ulcers and gangrene in the fingers and toes. Smoking makes this condition much worse.
The heart muscle can become scarred. Problems with the rhythm of the heart and heart failure can result.
Investigations
There may be a low hemoglobin (anemia), and an elevated serum creatinine (showing reduced kidney function). The specific antibody abnormalities are as shown in the table below.
A chest X-ray, a CT scan of the lung, and pulmonary function testing are used to evaluate scleroderma involvement in the lung.
Treatment
There is no good treatment for the skin disease, but there is evidence that penicillamine, cyclosporine, and methotrexate might be of minor benefit.
The heartburn (or acid reflux) is treated by decreasing the acid in the stomach with drugs that are proton pump inhibitors (e.g., Losec). The problems of moving food through the esophagus are treated with metaclopramide. Antibiotics are used to treat the diarrhea that results from the bacterial overgrowth.
A renal crisis, which includes deterioration of the kidney function and high blood pressure, is treated with ACE inhibitors (e.g., Captopril). There is some evidence that corticosteroids might cause a kidney crisis in someone with diffuse scleroderma, so these must be used with some care.
The lung involvement has been treated with cyclophosphamide, cyclosporine, and azathioprine.
Sjogren’s Syndrome
This condition can be associated with other connective tissue diseases or it can occur on its own. It causes dryness of the mouth, eyes, skin, and vagina, and can cause swelling of the salivary glands just in front of the jaw. Other symptoms of Sjogren’s syndrome include fatigue, arthritis, and a rash (usually on the lower legs, with tiny purple dots that may leave a brown discolouration). Patients may develop damage to the nerves; this shows up as numbness and tingling in the hands, face, and feet.
People with Sjogren’s syndrome have a small but increased risk of developing a tumour in the lymph system (lymphoma). If the parotid glands are enlarged, then they need to be monitored.
Investigations
The lab abnormalities include a reduction in the hemoglobin level (anemia), and a low white blood cell and platelet count. The ESR is elevated. Tests for the rheumatoid factor, ANA, and the Ro and La antibodies are usually positive.
Treatment
Treatment is directed at the symptoms. For the dryness of the eyes, artificial teardrops and tear gel are recommended. For the dryness of the mouth, drink lots of water and try chewing sugarless gum. With dryness in the mouth comes an increased problem with dental cavities, so you need to see your dentist regularly. Pain during intercourse due to vaginal dryness can be helped by using a lubricating jelly. Occasionally the eye doctor will put plugs in the ducts that drain the tears from the eye. This helps the dry eyes. Hydroxychloroquine (Plaquenil) may reduce joint pain and fatigue, and a pill called Salagen may increase saliva and tear production.
Polymyositis and Dermatomyositis
Polymyositis is an inflammatory disease of the muscles. It causes weakness of the proximal muscles (neck, back, chest, and shoulder and hip girdles), which will lead to difficulty getting out of bed or a low chair. It can also cause problems with breathing and swallowing.
Dermatomyositis is an inflammatory disease of the muscles and skin. It causes a rash, which is usually in sun-exposed areas. There may be a slightly raised, red, scaly rash over the knuckles and the bony areas of other joints. Rashes can occur on the eyelids, the V-area of the chest, and the shawl area of the nape of the neck and shoulders. There is a childhood form of this disease.
Both of these diseases can be associated with common cancers in adults. Appropriate investigation is recommended as the cancer can be found in an early, curable stage.
Investigations
The most common abnormality is an elevated level of CPK (which is an enzyme released from damaged muscle). Other tests used to make the diagnosis include electrical studies of the muscle (known as EMG, or electromyography) done by the insertion of a needle into the muscle, muscle biopsy, and MRI examination of the muscles.
Several autoantibodies are associated with inflammatory myositis. The anti-Jo1 antibody is associated with interstitial lung disease (inflammation and scarring of the lung) and a scaly rash on the hands.
Treatment
Most people will respond to moderately high doses of corticosteroids. If this fails or if the corticosteroid level cannot be reduced and eventually weaned, then methotrexate and azathioprine may be added. In stubborn cases that do not yield to treatment, intravenous gamma globulin is used. About 85 percent of patients will do reasonably well.
Psoriatic Arthritis
Psoriasis is a skin disease that commonly affects the scalp and the skin of the elbows and knees but it can occur anywhere on the skin. It appears as red patches with silvery to white scales. It is common in some families.
Psoriatic arthritis (PsA) is the arthritis that can occur with psoriasis. The joints can be swollen, reddish or purplish, tender, and warm. It commonly affects the small joints of the hands and feet but any joint can be involved, including those in the back and neck. It also affects the tendons and the attachments between tendons and bones (known as entheses).
Approximately 30 percent of patients with psoriasis have arthritis. Psoriasis occurs in 1–3 percent of the Canadian population, therefore psoriatic arthritis may occur in up to 1 percent of the population. This frequency is similar to that of rheumatoid arthritis. Psoriatic arthritis has been considered to be a mild form of arthritis, and so no specific treatments have been developed for this condition. However, recent studies have shown that the disease can be severe, with deformity and disability affecting about 20 percent of sufferers.
Psoriatic arthritis usually begins sometime after the onset of the psoriasis, but in 20 percent of cases the arthritis begins before the psoriasis, and in 10 percent of cases the psoriasis and arthritis begin at the same time. The common age of onset is between 30 and 50 years, and equal numbers of men and women get this disease. There is a juvenile form of psoriatic arthritis, and this will be discussed in Chapter 3, “Childhood Arthritis.”
Causes
Please see Appendix I, “Heredity and Environment.”
Symptoms
Most people have psoriasis prior to developing psoriatic arthritis. The affected joints become swollen, tender, stiff, and warm. Pain and swelling can occur in the tendons and ligaments at their point of attachment to the bone (e.g., at the back of the heel where the Achilles tendon inserts; see the illustration on page 39). The neck, back, and buttocks can also be affected. Arising from bed in the morning is difficult due to prolonged stiffness.
Psoriatic arthritis can show up in at least five different patterns, and some people will have a combination of types.
Type 1 primarily involves the DIP joints of the fingers and the toes. (See illustration on page 39.) Psoriasis is usually present in the nails adjacent to the affected joints, and typical changes to the nails would include nail lifting, pitting, and thickening. This type occurs in 10–15 percent of patients.
Type 2, arthritis mutilans, occurs in 5 percent of people with psoriatic arthritis. This is a severe destructive arthritis of the hands and feet in which the bones actually dissolve at the ends. The fingers and toes appear collapsed, which is called telescoping because it looks like you could pull or stretch the digit back to normal length in the same way as you would a telescope.
Type 3, symmetric polyarthritis, looks like rheumatoid arthritis except that the distal joints of the fingers and toes may be involved, the joints sometimes fuse, and the tendons of the hands are more commonly affected. (See illustration on page 19, which shows the joints that are likely to be involved here.) The rheumatoid factor is negative in the blood work.
Type 4, oligoarthritis, involves fewer than five joints (see illustration on page 39). It is associated with a “sausage digit” (when the tendon is markedly inflamed, it causes the finger or toe to look like a sausage). This pattern is seen about 40 percent of the time.
Type 5, where there is spinal involvement, affects the sacroiliac joints and the spine (from the low back to the upper neck). It is six times more likely to occur in a man than in a woman, and is similar to ankylosing spondylitis (which is discussed on page 41 of this chapter).
The incidence of other diseases such as Crohn’s disease and colitis is higher in people with psoriatic arthritis.
Investigations
There are no blood tests that specifically diagnose psoriatic arthritis. The rheumatoid factor is negative; if it is positive, then you may have both psoriasis and rheumatoid arthritis. The blood tests for inflammation (such as the C-reactive protein, the ESR, and the platelet count) may show high results.
The X-rays will likely be normal in early disease. Later, typical X-ray changes of the hands and feet for psoriatic arthritis may occur.
Treatment
Psoriatic arthritis can be treated with various medications and therapies, which we will discuss below.
Medication
NSAIDs are used to treat the pain and inflammation in psoriatic arthritis, and are particularly useful for treating pain and stiffness in the back and neck. If there is swelling in the joints, then a DMARD is required.
Sulfasalazine and gold have been shown to help psoriatic arthritis. There are concerns that Plaquenil or chloroquine may worsen the skin psoriasis.
Methotrexate is used to treat both the skin and joint disease of psoriatic arthritis. Many dermatologists request a biopsy of the liver to be sure that the methotrexate is not causing damage, but rheumatologists are less likely to require a biopsy. There is some evidence that liver damage from methotrexate may be more common when it is used to treat psoriasis than when it is used for rheumatoid arthritis.
Cyclosporine has been used for the skin and joint disease of psoriatic arthritis. Small clinical trials of the biological agents (etanercept and infliximab) have also shown a very good clinical response.
Sometimes, if the skin disease is well controlled, then the arthritis seems to improve. The use of ultraviolet light therapy combined with retinoids (Vitamin A products) is effective in treating the skin disease.
Ankylosing Spondylitis
Ankylosing spondylitis (AS) is an inflammatory arthritis that primarily affects the spine. The name is derived from the Greek words spondylos (spine), itis (inflammation), and ankylosis (inability to move). It is an ancient disease that has been seen in Egyptian mummies and dinosaurs. It usually begins during the period from the late teenage years to age 35, and affects seven times as many men as women. It is associated with the HLA B27 antigen 90 percent of the time; however, if you have the HLA B27 antigen, your chance of developing AS is still only 10 percent. If a first-degree relative (mother, father, sister, or brother) has AS, then your risk increases to 20 percent. (See Appendix I, “Heredity and Environment.”) AS is seen in association with psoriasis, Crohn’s disease, and ulcerative colitis.
This disease is characterized by pain and stiffness that are worst upon awakening in the morning and that last more than one hour. Physical activity usually improves the symptoms, which is not the case with the many other causes of low back pain. The first symptom will be pain in the buttock region and posterior thigh arising from the inflammation of the sacroiliac joint (see illustration below).
The pain usually progresses gradually over several weeks. Patients may feel unwell and tired. The inflammation will gradually spread up the spine to the ribs or chest wall and then to the neck. The sacroiliac joints will fuse as bone grows across them. There is no loss of function, however, as these joints barely move even when they are normal. As the joints in the chest wall, spine, and neck fuse, however, there will be loss of movement (see illustration on page 43).
Symptoms
AS progresses differently in different individuals. It tends to be less severe in women, which makes the diagnosis more difficult. How the disease progresses in the first five years usually predicts whether it will be mild or severe. In severe disease the spine will fuse, beginning with loss of range of motion in the lower back, progressing to decreased chest expansion, and being followed by loss of movement in the neck. The shoulders and hips may be involved as well. Some people will fuse in a bent position so that they are looking at their toes. This can be avoided by paying strict attention to posture and exercise. You will be less disabled if you are fused in an upright position.
Eye involvement is common with AS. The iris becomes inflamed (iritis or anterior uveitis), and the eye will be red, painful, and sensitive to light. This needs to be seen by a physician and treated.
Enthesitis refers to inflammation of the site where a tendon or ligament attaches to bone. This often occurs in AS, most commonly with the Achilles tendon at the back of the heel and the plantar fascia on the base of the heel. (See illustration on page 39.)
The heart valves (especially the aortic valve) are sometimes involved. For some reason, this was more common after the Second World War, when the troops returned from Europe. Some speculate that the difference may be due to the different strains of bacteria that the troops were exposed to. We rarely see this complication today. It is interesting that the valves of the heart are affected where the tendon-like material of the valve attaches to the heart muscle.
Lung involvement is rare. When it does happen, the upper segments of the lung become scarred or fibrous, but patients do not usually have symptoms from this. Patients with AS do, however, have restricted movement of the chest wall. The joints attaching the ribs to the spine can fuse, making it difficult if not impossible to expand the chest wall and clear secretions. It is especially important that people with this condition do not smoke.
Treatment
Treatment is aimed at controlling pain and stiffness, and preventing loss of range of movement and deformity.
The most common treatment for pain and stiffness is a high dose of a fairly potent NSAID. I have commonly used indomethicin or diclofenac at 150–200 mg daily. As many people do not tolerate one NSAID or another, it may be necessary to try a few to find one that works and is tolerated. I am frequently asked whether NSAIDs need to be taken on a regular basis. To control inflammation, they need to be taken for up to three or four weeks to achieve their full benefit. If you have no pain and no stiffness, then the medication can sometimes be stopped. However, if there is pain or stiffness, then it is important to continue taking the NSAID so that you will move better and preserve range of motion.
Several DMARDs have been used to treat ankylosing spondylitis. Salazopyrin helps the peripheral joint disease but not the spine joint disease. Methotrexate has been tried, but there are no good studies demonstrating its efficacy in this disease.
Pamidronate is an intravenous bisphosphonate used to treat osteoporosis and cancer that has spread to the bone. It has been tried in AS with mixed results, but studies are presently ongoing.
Early studies and experience with the biological agents, etanercept and infliximab, are very promising for the treatment of ankylosing spondylitis. The dosage of infliximab is greater in AS than it is in rheumatoid arthritis, and these drugs are very expensive.
Self-Help
A daily exercise program is essential to maintain range of motion and posture, and will also help to control pain and stiffness. It is important to strengthen the antigravity muscles (front of the thighs, buttocks, and shoulders) and the abdominal muscles in order to maintain an upright posture. Extension exercises are important (such as lying on your stomach and doing press-ups for 20 minutes). A cardiovascular workout will improve your general conditioning and your chest expansion. As you age and the disease changes, you may find that your exercise program needs to be modified. It is important to have the help of a good physiotherapist who is knowledgeable about AS.
Recreational activities are important to all of us. If you have AS, you should avoid activities that keep you in a flexed position (such as bowling, curling, cycling, and hockey). If these activities are very important in your life, however, then balance them with other activities to increase flexibility and promote extension (such as tennis, golf, and tai chi). Please avoid contact sports and play hockey only in a no-checking league (if side-checked, you may be seriously injured due to the loss of flexibility of the spine and supporting tissues).
Keep a straight posture whether you are standing, walking, sitting, or lying down. Patients are encouraged to sleep on their side (but not to curl into a ball), or to sleep on their back with one flat pillow. You should have a firm mattress or a board under the mattress.
A patient with AS should never allow a chiropractor or anyone else to manipulate the spine. In AS, the joints, ligaments, and tendons supporting the spine are fused with bone. Manipulation may cause a fracture through these unmovable bony structures.
Use your medications regularly when the symptoms of inflammation are present. Smoking is absolutely to be avoided: because of the chest wall restriction, smoking will damage the lungs even more than it would in an otherwise healthy person.
As the onset of this disease is in the late teenage and the early adult years, career counselling is an important consideration. In general, you want to consider a career with varied daily activities: someone who must sit all day will be stiff and sore, and heavy physical activity may aggravate the pain. Think carefully before choosing a career. At work, you should stand and stretch every hour. You should avoid driving for prolonged periods of time, but if this is necessary, then stop and stretch frequently.
Reactive Arthritis
This refers to an arthritis that is precipitated by an infection. Most of the time it refers to a triad of symptoms that includes arthritis, urethritis (discharge from the penis and pain when urinating), and conjunctivitis (a red eye). This was previously called Reiter’s syndrome but this term is no longer used since it was felt to be inappropriate to honour Klaus Reiter as he was a Nazi and guilty of war crimes in concentration camps.
Reactive arthritis may also refer to any arthritis that develops because of an illness caused by an infectious agent. For example, it may occur after a strep throat. The organism is not found in the joint; it is the body’s immune response to the infection that causes the arthritis. The triad of arthritis, conjunctivitis, and urethritis is caused by a urogenital infection, a chlmydial infection, or an infectious diarrhea.
A better understanding of this disease occurred as a result of an unfortunate incident in the 1980s during the Pope’s visit to Canada. A large contingent of the Ontario Provincial Police ate sandwiches that were shipped from Montreal to Toronto in an unrefrigerated truck. As you might anticipate, there was a huge outbreak of salmonella poisoning in the police force, and some individuals went on to develop a reactive arthritis. These individuals were genetically predisposed: they carried the HLA B27 antigen, the same antigen that predisposes you to develop ankylosing spondylitis. Some of the officers developed a chronic deforming arthritis, whereas others had an arthritis only for a few months. See the table below for a list of infections that cause a classic reactive arthritis.
This condition occurs most frequently in young adults aged 20–40. It is more common in males if the infectious source is urogenital or sexually acquired, and equally common in males and females if it is from food poisoning (such as with salmonella or shigella).
Symptoms
Most people develop symptoms about one to four weeks after the infection (which can be very mild or can even pass totally unnoticed).
The arthritis favours the lower limbs. The same joints will not be affected on both sides of the body, and usually fewer than five joints are involved. The inflamed joints are red, swollen, and very tender. The fingers and toes may swell up like sausages (dactylitis), which means that all of the tendons and joints of the digit are inflamed. The point where a tendon connects to bone can become inflamed (this is called enthesitis), and therefore Achilles tendonitis and plantar fasciitis are common symptoms. The spine and sacroiliac joints often become inflamed. The typical changes of sacroiliitis and spondylitis will be seen on X-rays in people with longstanding disease.
Other features of reactive arthritis include low-grade fever and weight loss. The front of the eye can become inflamed with either conjunctivitis or the more serious iritis. A rash can occur on the penis (circinate balanitis) and on the soles of the feet (keratoderma blenorrhagicum).
The genital and urinary tracts may be affected by infectious or sterile urethritis, prostatitis, cystitis (inflammation in the bladder), or salpingitis (inflammation in the Fallopian tubes).
Investigations
The diagnosis of reactive arthritis is based on a history and physical examination, and you therefore need to be seen by a physician who is familiar with this condition.
If reactive arthritis is suspected, then the joint fluid should be tested if possible to be sure that there is no an infection in the joint. Swabs should be taken from the man’s penis or woman’s cervix and tested for chlamydia and gonorrhea. A stool sample should be sent to the lab for bacterial identification.
The blood tests will show an elevation in the CRP and ESR, suggesting an active inflammatory process. Sometimes, blood tests will be ordered to rule out other types of arthritis.
X-rays of the affected joints done early in the disease are not likely to be helpful as it takes some time for them to become abnormal with the typical changes of reactive arthritis.
HLA B27 is not routinely tested as some people will develop reactive arthritis in the absence of B27, and others who are B27-positive can develop other types of arthritis.
Treatment
Reactive arthritis is not a joint infection that would respond to antibiotics. Instead, the immune system is reacting to the infection in the bowel or genito-urinary tract. If an infection is identified then it should be treated with antibiotics, but the antibiotic is unlikely to affect the course of the arthritis.
The arthritis is usually treated with rest and sometimes with splinting. NSAIDs are used at high doses (e.g., indomethacin at 150–200 mg/day). If this does not work, then an injection of corticosteroids into the joint may help. Analgesics can be used for pain control.
This disease will usually go into remission after two to six months. Approximately 20 percent of patients will develop a chronic arthritis and need to be treated with DMARDs. Salazopyrin and methotrexate have been used with some success.
The syndrome can recur with reinfection.
Crystal Induced Arthritis
The two most common forms of arthritis caused by crystals are gout (from urate acid crystals) and pseudogout or CPPD (calcium pyrophosphate dihydrate deposition disease, which is caused by calcium pyrophosphate dihydrate crystals). Calcium hydroxy apatite may cause a marked destructive arthritis of the shoulder and be associated with osteoarthritis and calcific tendonitis (e.g., calcium deposits on the shoulder).
Gout
Gout is caused by there being too much uric acid in the blood, which may then crystallize in the joint. The excess of uric acid is usually caused by the body either producing too much uric acid and/or not passing enough through the kidneys into the urine. An elevated uric acid level can cause arthritis, but it can also cause kidney stones in high excreters of uric acid. In addition, it may lead to some decline in the function of the kidney if the level in the blood is very high over a prolonged period. (Asymptomatic hyperuricemia is a high level of uric acid in the blood that does not need to be treated. The exception to this is if the level is extremely high or if you are receiving chemotherapy for cancer.)
Gout was first described in the fifth century by Hippocrates, although it had been recognized by the Babylonians. The name comes from the Latin gutta (drop), because in the thirteenth century it was thought that gout was caused by a drop of evil humour. Gout has been referred to as “the disease of kings,” since it was thought that overindulgence led to the development of gout. Certainly, in a predisposed person, alcohol and a diet rich in purines (shellfish, organ meats) will cause an attack.
Van Leeuwenhoek, the inventor of the microscope, first described the urate acid crystals, which looked like small needles. In fact, when they are swallowed up by the white blood cells, their sharp ends rupture the cells, leading to the release of chemicals that stimulate the inflammatory process.
People usually develop gout between the ages of 40 and 50. Gout occurs more commonly in men then women. Some famous people who had gout include Charles Darwin, Sir Isaac Newton, Michelangelo, and Benjamin Franklin. The risk factors for the development of gout include a family history of gout, prolonged use of a diuretic (medication to cause fluid loss), other drugs, alcohol ingestion, high cholesterol, obesity, kidney failure, widespread psoriasis, and low thyroid hormone production.
Symptoms
Usually the first attack of gout comes on suddenly in the middle of the night. Patients will awaken with such severe pain that they cannot even stand the weight of a bedsheet on the joint. The joint most commonly affected is the big toe, because this is the coldest joint and the crystal forms more readily at a low temperature. Other commonly affected joints include the arch of the foot, the ankle, the knee, and the wrist. The joint will be red, swollen, and excruciatingly painful. An attack in the joint lasts several days if treated, and three to four weeks if untreated.
Events that might trigger an attack of gout include alcohol ingestion, increased purine diet (shellfish, organ meats), trauma, surgery, certain drugs, radiation, dehydration, and hemorrhage. Once you have had one attack of gout, it is likely that you will have another, although it may be years later. As time goes by, the attacks become more frequent and last longer, and begin to affect more than one joint at a time. When the attacks begin to become more frequent, it is advisable to begin a drug to lower the uric acid level in the blood and prevent further attacks.
If the disease is not treated, a period of time will pass during which you will not have any attacks. However, some patients then develop chronic tophaeous gout, where the crystals form deposits (tophi) under the skin of the elbows, the ears, the top of the foot, the Achilles tendon, or the joints, causing a destructive arthritis. The tophi can open and drain if untreated. Chronic gout can damage all the peripheral joints and can be mistaken for rheumatoid arthritis. The tophi can be mistaken for rheumatoid nodules. Chronic tophaeous gout can be completely avoided if treated properly. Elderly women who have never had an attack of gout sometimes get tophi over the finger joints where they have changes of osteoarthritis.
Treatment
Gout is a treatable disease, but the treatment has to be done correctly. An acute attack of gout should be treated with ice, rest, and high doses of an NSAID. The usual drug is indomethacin at 150–200 mg per day for three to seven days. There is no reason that other NSAIDs cannot be used. There are times when an NSAID is not a good choice, and other alternatives are available. (For instance, if a patient has had a stomach ulcer or bleed or if the kidney is not functioning well, then an NSAID should be avoided.) Colchicine is an old drug that has been given for acute gout since the mid-1800s. It must be taken every one to two hours as a pill until you are better or until you get diarrhea. Usually, a person would not take more than six doses in a row. Occasionally the best treatment is to inject the joint with corticosteroids or, if many joints are involved, to give a short course of corticosteroids by mouth, intramuscularly or intravenously.
During an acute attack, drugs to lower the uric acid level in the blood should not be started; however if you are already on one of these, then continue it but do not change the dosage. These drugs would include allopurinol, sylfinpyrazone, or probenecid. If you start one of these drugs, increase the dosage, or stop the drug during an acute attack, then you will prolong the attack and make it more severe.
Once you start a drug that lowers the uric acid level, you must take it for the rest of your life. Since a second attack often doesn’t come until years after the first attack, I usually do not start medications to lower the uric acid until the attacks become more frequent. The uric acid level must be decreased to at least half the upper limit of the normal range for uric acid in the blood to prevent attacks and dissolve the tophi under the skin. This value will differ in different labs. Once the uric acid is lowered you will have no further attacks, and the deposits under the skin will dissolve. However, as the uric acid levels are being lowered, there may be an increase in the frequency of gout, which can be prevented by small daily doses of an NSAID or colchicines.
There are several medications that lower the uric acid level. Allopurinol causes the body to make less uric acid. The other drugs, probenecid and sylfinpyrazone, lower the uric acid level by increasing the amount that passes out of the body in the urine (these drugs cannot be used if you have a history of kidney stones, reduced kidney function, or if you excrete too much uric acid in the urine to begin with). Please see Chapter 7, “Medications.”
Diet alone will not control gout. (Please see Chapter 11, “Diet and Nutrition.”) If you have a high uric acid level, then alcohol or a diet high in purines may cause an attack.
Gout can be treated and all further attacks prevented if you take your medications correctly. This means that, for the rest of your life, you will need to take a drug to maintain a low uric acid level. If you stop, the gout will come back: it may take a year or two, but it will return. Often, the attacks of gout may continue even with medication until the tophi under the skin have disappeared, so treatment may take some patience.
CPPD
Calcium pyrophosphate dihydrate deposition disease (CPPD) is a form of arthritis caused by a calcium salt. It is also referred to as pseudogout, because an acute attack causes an acutely painful, swollen joint like gout does. The arthritis is caused by the release of the calcium crystals into the joint. The crystals are engulfed by white blood cells, and this starts an inflammatory response and an arthritis. X-rays usually show calcification of the cartilage (referred to as chondrocalcinosis).
This form of arthritis is often seen in people with osteoarthritis. It is more common with increased age, and by age 90, chondrocalcinosis is seen in 50 percent of X-rays.
Symptoms
CPPD should be suspected if any of the following common symptoms are noticed:
- A joint may be acutely painful, tender, and swollen in an older person. The usual joints to be affected are the knees and the wrists.
- Osteoarthritis symptoms might be experienced, except that the symptoms are also in joints that are not usually affected by primary osteoarthritis (such as the MCP joints of the hands, or the wrists or shoulders).
- Rheumatoid arthritis might appear to be the problem, with chronic swelling of the knuckles (MCP joints), wrists, and knees, but the rheumatoid factor is negative.
- You might have a severely destroyed joint that is out of keeping with typical arthritis.
Diagnosis
The diagnosis is often suggested by the history and physical exam. The X-rays can be helpful to confirm the diagnosis, as they may show typical changes. A swollen joint should be aspirated and the fluid sent for analysis. CPPD crystals can be seen under the microscope. It is very important that the fluid also be sent for culture as an infection and CPPD can sometimes coexist.
If a diagnosis of CPPD is made, then investigations are done to rule out other diseases that can accompany it. These include hemachromatosis, a disorder of iron overload that will damage the liver and other organs unless it is treated with regular phlebotomies (blood-letting) to normalize the iron. CPPD can also be seen in association with hyperparathyroidism (an overproduction of parathyroid hormone, which leads to high calcium levels in the blood) and hypomagnesemia (low magnesium levels in the blood). These are all investigations with blood work.
Treatment
The usual treatment is to take an NSAID. DMARDs are generally not used for this condition. An acute attack in one or two joints may be treated with an injection of corticosteroids, but this should only be done once it is certain that there is no infection in the joint. Colchicine has been used to try to prevent recurrent attacks, but this is only moderately successful. NSAIDs taken on a regular basis may also prevent recurrent attacks.
Most people may have recurrent attacks of joint swelling but usually the attacks are short-lived and spread apart in time. The disease is not progressive like rheumatoid arthritis. If there is joint damage, the outcome is similar to osteoarthritis. Diet does not seem to play a role in the disease, and exercise is important to maintain strength and range of movement.
Polymyalgia Rheumatica and Temporal Arteritis
Polymyalgia rheumatica (PMR) is a disease that is seen after the age of 50 and becomes more common as people get older. This condition can be associated with temporal arteritis (TA), which is an inflammation of the temporal artery (at your temple). Approximately 10–30 percent of patients with PMR will develop TA; however, approximately 60 percent of people with TA will have associated PMR.
The symptoms of polymyalgia rheumatica are severe stiffness and pain upon awakening, and sometimes the pain will wake you from sleep. The shoulder and hip girdles and the neck are all affected, but muscle weakness is not a feature of this condition. There may be arthritis, and patients may have fatigue and feel unwell. However, if there is swelling in the small joints of the fingers in a symmetric pattern, then the diagnosis may be late-onset rheumatoid arthritis.
With temporal arteritis, the symptoms include a headache and scalp tenderness. There may be pain in the jaw muscles when chewing food that is relieved by stopping (this is called jaw claudication). There may be pain down the arms that is made worse by using the arms. There can be swelling, pain, and tenderness of the temporal artery. The major complication of this condition, if not treated, is blindness.
When I first started my practice I had the privilege of seeing a woman in her nineties who came to me with the most severe headache of her life. She had a red, hot, tender artery over the left temple. Her ESR (test for inflammation) result was very high, and the biopsy suggested temporal arteritis. This woman had survived the calamitous December 6, 1917, Halifax explosion, when two ships collided in Halifax harbour; one of them, the Mont Blanc, was full of munitions. Many Haligonians went to their windows and watched the fire on board the Mont Blanc, unaware that it was about to explode and create the largest man-made explosion before Hiroshima. The explosion shattered windows, and shards of glass pierced the eyes of hundreds. My patient was one of those people, and had had no vision in her right eye since she was a teen; she said that she would rather die than lose her remaining vision. High doses of prednisone took care of the symptoms very well, and fortunately, her vision was preserved.
Diagnosis
The diagnosis of polymyalgia rheumatica is made based on history and examination. The blood will usually show a marked elevation of the ESR. The diagnosis is often confirmed by giving the patient 15–20 mg of prednisone per day: if the diagnosis is correct, then the symptoms will usually disappear in 24–48 hours. The response is dramatic.
The diagnosis of temporal arteritis is made with a history and a physical examination. The ESR is very high. A biopsy is performed of the temporal artery, which should show characteristic inflammatory changes. Sometimes the biopsy is negative, however, because there can be skip lesion (areas of the artery are not involved) and the inflamed area may have been missed in the biopsy.
Treatment
The treatment of polymyalgia rheumatica is low doses of prednisone (usually 15–20 mg once daily). The symptoms should resolve promptly. If the prednisone is weaned too quickly, however, the symptoms will recur. Most individuals require treatment for at least one to two years.
The temporal arteritis will go away after several years. Normally the disease is treated with prednisone at 50–60 mg per day, and when the ESR normalizes, the prednisone is weaned gradually over the course of a year or so.
There are many forms of arthritis, and in this chapter I have attempted to highlight a few of the more common types. It is important to understand what type of arthritis you may have, and how it should be most appropriately treated. If you have an inflammatory arthritis such as rheumatoid arthritis, then prompt diagnosis and treatment will lead to the best long-term results.
