Arthralgia is a common nonspecific symptom that can occur in any chronic illness, but some patients with HiV develop attacks of acute severe joint pain without any swelling. These attacks of acute arthral-gia often subside spontaneously over a few days. Several types of arthritis may occur. The most common is a type of arthritis that overlaps with reactive arthritis and Reiter's syndrome. Asymmetrical swelling of large joints such as knees and ankles and inflammation of tendons such as the Achilles tendon and of sites where tendons join bone (enthesi-
tis) may occur. These are sometimes accompanied by other symptoms of Reiter's syndrome such as urethritis and conjunctivitis. Experts disagree whether the incidence of this type of arthritis is really increased by HIV infection. Some believe that HiV infection is often a marker for high-risk sexual behavior, and therefore the incidence of Reiter's syndrome would be expected to be higher because of this and not because of the HiV infection. The strongest argument against this point of view comes from studies performed in Africa where Reiter's syndrome and reactive arthritis were uncommon before the HiV epidemic but are now common medical problems, almost always occurring in patients with HiV infection. Reiter's syndrome in patients who do not have HiV infection is usually triggered by a clearly diagnosed infection such as diarrhea caused by salmonella or urethritis caused by chlamydia. However, in HiV-associated arthritis a specific trigger is seldom identified. HiV-associated arthritis is usually treated with nonsteroidal antiinflammatory drugs to control pain and swelling. in most patients the arthritis settles over a few months, but in some it persists. There are no large scientific clinical trials to provide information how best to treat this type of arthritis. Clinical experience suggests that some patients with persistent arthritis respond to sulfasalazine, a drug also used to treat inflammatory bowel disease and rheumatoid arthritis, or to HYDROXYCHLOROQUINE. METHOTREXATE is avoided because some patients have developed Kaposi's sarcoma, a type of cancer that occurs more often in patients with HiV infection. if a single joint is particularly inflamed and bacterial infection of the joint has been excluded, it may respond to an intra-articular corticosteroid injection.
A handful of patients have developed a type of arthritis that is similar to rheumatoid arthritis with symmetrical inflammation of the small joints of the hand. it is not clear if this is just coincidence and rheumatoid arthritis has developed in a person who is also infected with HiV or if this is part of the spectrum of arthritis associated with HiV.
Psoriatic arthritis Severe psoriasis and psori-atic arthritis can occur for the first time after a person is infected with HiV. The rash is often pustular. The arthritis is typical of psoriatic arthritis with asymmetrical involvement of large joints such as the knee or smaller joints of the fingers or toes, causing sausage digits. Psoriatic arthritis in patients with HIV infection can be difficult to treat. Methotrexate, a drug that would usually be used to treat severe psoriatic arthritis, is avoided because it increases the risk of infection and may increase the risk of Kaposi's sarcoma.
Infective arthritis Joint infections are more common in patients with HIV infection and are most commonly caused by a bacterium, Staphylo-coccus areus, the same organism that is often the cause of infective arthritis in patients without HIV infection. The symptoms and treatment are similar. Patients with HIV infection, because of immuno-suppression, can have joint infection caused by unusual organisms, including fungi and mycobac-teria. Intravenous drug users with HIV infection are more likely to get infected joints.
Avascular necrosis Some people who are infected with HIV get avascular necrosis (AVN) in bones, and this causes acute pain and eventually weakening of the bone. Scientists do not know how HIV causes AVN.
Vasculitis Most types of vasculitis have occurred in patients with HIV. Whether this is just coincidence or in some way related to the virus is not clear.
Sjögren's syndrome Patients with HIV infection can develop a condition similar to Sjögren's syndrome with enlargement of the salivary glands and symptoms of dry mouth and eyes. Sjögren's syndrome is caused by proliferation of CD4 lymphocytes in salivary glands and other tissues. The syndrome associated with HIV is caused by CD 8 lymphocytes and is sometimes called diffuse infil-trative lymphocytosis syndrome (DILS). It can involve the lungs and cause difficulty breathing.
SLE overlap syndrome HIV infection does not cause SLE. In fact, because of the immunosuppression it causes, it may protect against it. Because many of the symptoms of HIV and SLE overlap, the two conditions can be mistaken. For example, fever, loss of weight, arthralgia, arthritis, facial rash, hair loss, glomerulonephritis, leukopenia, thrombocy-topenia, seizures, coma, pneumonia, and a positive ANA blood test can occur in both. A false positive ELISA HIV test can occur in patients with SLE, but more specific tests for HIV are negative.
Myositis and rhabdomyolysis HIV is associated with several types of muscle disease. Myalgia (muscle aching) is very common but occurs with many chronic conditions. Severe loss of muscle mass occurs with advanced AIDS and in some countries was called slim disease. An illness very similar to DERMATOMYOSITIS AND POLYMYOSITIS with weakness of proximal muscles and elevated muscle enzymes can occur. Some of the drugs used to treat HIV can affect muscles, for example zidovudine (AZT) can cause myositis. Muscles are remarkably resistant to infection, but in patients with HIV infection abscesses can occur in big muscles such as those of the thigh (pyomyositis). Rhabdomyolysis with severe muscle damage, very high levels of muscle enzymes, dark urine caused by a protein called myoglobin (the result of muscle breakdown), and impaired kidney function have occurred in approximately 20 patients with HIV. Sometimes recognized causes of rhabdomyolysis such as prolonged seizures or drug therapy have been present, but in some cases HIV infection has been the only predisposing cause.
Fibromyalgia chronic pain, fatigue, and fibromyalgia are symptoms that occur more often in HIV infection and many other chronic illnesses.
Hypertrophic pulmonary osteoarthropathy (HPOA) chronic bacterial infection, particularly lung infection, can cause swelling of the ends of the fingers (clubbing) and pain and swelling at the end of long bones close to joints like the knee and wrist. Treatment of the infection improves the rheumatic symptoms.
hypermobility syndrome The occurrence of musculoskeletal symptoms in a hypermobile patient who does not have another recognizable generalized rheumatic disease. The exclusion of another rheumatic disease is important because many of the manifestations of hypermobility can also occur in people who do not have hypermobil-ity, and it is more the number and wide range of problems that typifies this syndrome. Despite hypermobility first being described by Hippocrates more than 2,000 years ago, it is a difficult concept for many people and remains poorly understood and underdiagnosed. Hypermobility is a feature of many inherited disorders of connective tissue such as Marfan's and Ehlers-Danlos syndromes. These are considered to be separate from what is sometimes termed benign hypermobility syndrome.
unusual looseness of the ligaments can be found, at least in one or two joints, in up to 10 percent of the population. It is particularly common in children and diminishes rapidly during childhood and then more slowly in adulthood. In general, African Americans have a greater range of joint movement than caucasians, and Asians a greater range than African Americans. People with hypermobility will frequently have been called double-jointed as children and may make career choices as a result of their condition. All contortionists are hypermobile as are many ballet dancers. It should be emphasized that not everyone with hypermobility will develop problems because of it (see definition above).
Patients with the inheritable connective tissue diseases Ehlers-Danlos and Marfan's syndromes have reasonably clear-cut abnormalities in the makeup of their collagen or fibrillin (connective tissues). Very likely hypermobility syndrome has similar as yet undefined causes. Hypermobility syndrome often runs in families, and some differences in the make up of collagen have been shown in these families. The connective tissues form most of the substance of tendons, ligaments, and cartilage as well as supporting structures such as the skin, arteries, and heart valves. It is therefore these organs that show weakness or abnormal function if the connective tissue is slightly abnormal.
The problems experienced by people with hyper-mobility syndrome can be divided into four groups.
• More traumatic and overuse injuries occur than average. These include partial or complete tendon or ligament tears, especially around the shoulder and ankle, and injury with inflammation at the attachment of ligaments and tendons to bone (enthesitis). Back pain is also common.
• The ligaments are looser than normal, thus joints may become unstable. This can lead to dislocation, either complete or partial (known as subluxation). commonly affected joints are the shoulder, kneecaps, metacarpophalangeals, and jaw joint. Flat feet may be a manifestation of subluxed joints.
• A chronic arthritis develops. Usually this is osteoarthritis, which is likely to develop earlier than average. A few patients also develop a mild inflammatory arthritis (see arthritis for the difference) that is secondary to the repeated trauma but is probably often misdiagnosed. Many people with hypermobility syndrome suffer from joint and muscle pain with no obvious abnormalities to be found on examination. if the hyper-mobility is not recognized, these people may be told there is nothing wrong with them and frustration and depression is then understandably common.
• organs outside the musculoskeletal system may be affected. The skin may be thin and stretchy and develop striae (scarlike lines similar to those that women develop on their abdomens after pregnancy). Prolapse of the mitral valve may occur. This allows blood to leak back from the main pumping chamber in the heart (the left ventricle) to the collecting chamber (left atrium). individuals with hypermobility syndrome probably have more abdominal hernias, rectal and vaginal prolapse, stress fractures, and the head of the thighbone pushing through the pelvic bone (protrusio acetabuli). A number of patients have a physical appearance similar to patients with Marfan's syndrome (marfanoid habitus).
Many different sets of criteria for diagnosing hyper-mobility syndrome are still used, although there have been international efforts to form an agreed method. The commonly used major criteria are a Beighton score (see below) of 4/9 or greater and joint pain in four or more joints for longer than three months. Marfan and Ehlers-Danlos syndromes must be excluded first. Beighton described a series of tests that led to a scoring system for generalized hypermobility. These are positive if the little finger bends back more than 90°, the thumb can be bent back to touch the forearm, the elbows and knees bend backward (hyperextend) excessively (score 1 for each side), and if the hands can be placed flat on the ground with the legs straight (score 1).
if either four out of nine Beighton criteria or pain in four joints are not present, minor criteria can be used to make the diagnosis, but more are needed. These include a Beighton score of less than 4, more than one joint dislocation, skin striae, recurrent tendon and ligament injuries, Marfan-like body build, mitral valve prolapse, and hernias or rectal prolapse.
The first step in managing hypermobility syndrome is actually to make the diagnosis and provide information about it. Many patients have suffered unexplained pain for some time, and understanding their condition can give considerable relief. An assessment of the impact on the patient's life should be made and sensible advice concerning career and recreational choices given. Treatable complications such as tendinitis are identified and treated as they occur. Special caution should be taken before embarking on surgical procedures due to poor tissue healing and the frequently disappointing results from surgery.
ideally, the patient should be referred to a physical therapist with special experience in hyper-mobility, although such a person may not always be available. Physical therapy involves teaching the patient to recognize his or her (greater-than-normal) range of movement and do regular stretches through this range. Muscle strengthening, with most exercises done in the middle of the range of movement, and proprioceptive exercises (such as standing on a wobble board) have been shown to reduce pain in patients with hypermobil-ity syndrome. Many patients with generalized hypermobility have flat feet, and appropriate use of orthotics is important. Acute pain is treated with analgesics and NSAIDs, and chronic pain may require use of low doses of antidepressants and/or
The outcome for individual patients will vary considerably. Some people may be troubled by so-called growing pains as children and then grow out of it as their tissues tighten up with aging. others with severe hypermobility may experience many of the problems listed above; develop hip, knee, and back problems in early adulthood; and go on to get early osteoarthritis.
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