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

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Medical Editor: William C. Shiel Jr., MD, FACP, FACR

• What is Still's disease?
• What causes Still's disease?
• How does Still's disease fit in with juvenile rheumatoid arthritis?
• What are symptoms and signs of Still's disease?
• How is Still's disease diagnosed?
• What is the frequency of Still's disease and its features?
• What is the outlook with Still's disease?
• How is Still's disease treated?
• What research is being done on Still's disease?
• Still's Disease At A Glance

What is Still's disease?

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).

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 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.