Systemic-onset juvenile idiopathic arthritis
Systemic juvenile idiopathic arthritis
Systemic onset juvenile rheumatoide arthritis
Still's disease, a disorder featuring inflammation, is characterized by high spiking fevers, evanescent (transient) salmon-colored rash, and/or arthritis. Still's disease was first described in children, but it is now known to occur, much less commonly, in adults (in whom it is referred to as adult-onset Still's disease).
Still's disease usually begins with systemic (body-wide) symptoms. Extreme fatigue can accompany waves of high fevers that rise daily to 102 degrees F (39 degrees C) or even higher and rapidly return to normal levels or below. Fever spikes often occur at nearly the same time every day. A faint salmon-colored skin rash characteristically comes and goes and does not itch. Poor appetite, nausea, and weight loss are common. There is also commonly swelling of the lymph glands, enlargement of the spleen and liver, and sore throat. Some patients develop inflammation around the heart (pericarditis) and lungs (pleuritis), with occasional fluid accumulation around heart (pericardial effusion) and lungs (pleural effusion). Arthritis, with joint swelling, often occurs after rash and fevers have been present for some time. Although the arthritis may initially be overlooked because of the impressive nature of the systemic symptoms, everyone with Still's disease eventually develops joint pain and swelling. This usually involves many joints (polyarticular arthritis). Any joint can be affected, although there are preferential patterns of joint involvement in Still's disease.
There have been a number of schools of thought regarding the cause(s) of Still's disease. One concept is that Still's disease is due to infection with a microbe. Another idea is that Still's disease is a hypersensitive or autoimmune disorder. In fact, the cause of Still's disease is not yet known.
Still's disease is diagnosed purely on the basis of the typical clinical features of the illness. These features include high intermittent fever, joint inflammation and pain, muscle pain, faint salmon-colored skin rash, swelling of the lymph glands or enlargement of the spleen and liver, and inflammation around the heart (pericarditis) or lungs (pleuritis). Persistent arthritis (arthritis lasting at least six weeks) is required to make a firm diagnosis of Still's disease. Other diseases (especially infections, cancers, and other types of arthritis) are excluded. Many patients with Still's disease develop markedly elevated white blood cell counts, as if they have a serious infection, but no infection is found. Low red blood counts (anemia) and elevated blood tests for inflammation (such as sedimentation rate and C-reactive protein) are common. However, the classic blood tests for rheumatoid arthritis (rheumatoid factor) and systemic lupus erythematosus (antinuclear antibodies) are usually negative.
25% of cases progress to severe destructive arthritis.
Still's disease can cause serious damage to the joints, particularly the wrists. It can also impair the function of the heart and lungs. Treatment of Still's disease is directed toward the individual areas of inflammation. Many symptoms are often controlled with anti-inflammatory drugs such as aspirin or other nonsteroid drugs (NSAIDs). Cortisone medications (steroids) such as prednisone are used to treat more severe features of illness. For patients with persistent illness, medications that affect the inflammatory aspects of the immune system are used. Medications now being used are analogous to the classic "second-line" therapies used for patients with rheumatoid arthritis. These include gold, hydroxychloroquine (Plaquenil), penicillamine (Cuprimine, Depen), azathioprine (Imuran), methotrexate (Rheumatrex, Trexall), and cyclophosphamide (Cytoxan).