Still's Disease (Systemic-Onset Juvenile Rheumatoid Arthritis)

What is Still's disease?

Still's disease is a disorder featuring inflammation that 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).

What causes 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.

How does Still's disease fit in with juvenile rheumatoid arthritis?

Still's disease is one type of juvenile rheumatoid arthritis (JRA) and is also known as systemic-onset JRA. By "systemic" it is meant that along with joint inflammation it typically begins with symptoms and signs of systemic (body wide) illness, such as high fevers, gland swelling, and internal organ involvement. Still's disease is named after the English physician Sir George F. Still (1861-1941).

What are symptoms and signs of 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 of the lungs (pleuritis) or around the heart (pericarditis) with occasional fluid accumulation around the lungs (pleural effusion) or heart (pericardial 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.

How is Still's disease diagnosed?

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 of the lungs (pleuritis) or around the heart (pericarditis). Persistent arthritis (arthritis lasting at least 6 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 none 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, ANA) are usually negative.