Still's Disease
(Systemic-Onset Juvenile Rheumatoid Arthritis)
Medical Editor: William C. Shiel Jr., MD, FACP, FACR
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.
Next: How is Still's disease diagnosed? »
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