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Still's disease
From Wikipedia, the free encyclopedia
| ICD-10 | M06.1, M08.2 |
|---|---|
| ICD-9 | 714.30 |
Still's disease is a form of juvenile idiopathic arthritis, characterized by high spiking fevers and transient rashes, named after the English physician Sir George Frederic Still (1861-1941). The disease was first discovered in children, but now it is also known to occur, less commonly, in adults in whom it is referred to as adult-onset Still's disease.
There are several theories about the cause of Still's disease. It has been suggested it may be caused by a microbacterial infection or that it is an autoimmune disorder. However, the cause of Still's disease remains unknown.
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Patients with Still's disease usually have systemic symptoms. Usual symptoms include:
- waves of high fevers that rise to 40 °C (104 °F) which may be accompanied by extreme fatigue
- A faint transient non-itching salmon-colored skin rash can also be observed.
- Flu like pain throughout the body,
- muscle pain
Other signs and symptoms include:
- swelling of the lymph glands (lymphadenopathy)
- enlargement of the spleen (splenomegaly) and liver (hepatomegaly)
- sore throat
- pleurisy and pericarditis -- inflammation of the pleura (the lining around the lungs) or the pericardium (the lining around the heart) with fluid accumulation.
- Although the arthritis may initially be overlooked because of the other symptoms, children with Still's disease eventually develops pain and swelling in several joints. Though any joint can be affected, some joints (like the wrists) are more likely to be affected by the disease than others. In some patients with ""Adult-onset"" Still's Disease, pain in the joints arthralgias may be predominant over swelling and typical signs of arthritis.
Because the disease presents in many different ways, diagnosis is difficult. It is often a laborious process consisting of the elimination of other diseases. It is diagnosed on a basis of clinical features of the illness, with the results of a number of common tests combined. Persistent arthritis (lasting at least 6 weeks) is frequently a symptom. A rash is frequently visible on the body, and the patients usually suffer from high, spiking fevers. Blood tests frequently indicate elevated white blood cell counts, suggesting there is serious inflammation. Also, low red blood cell counts (anemia) are common. Typically, elevated blood tests indicate in high sedimentation rates, an indicator of inflammation. Other indicators of inflammation may also be elevated, including CRP (c-reactive protein) and ferritin levels. However, the classic blood tests for rheumatoid arthritis and systemic lupus erythematosus are usually negative.
The fever and most of the other symptoms tend to run their course within several months. However, the arthritis can become a long-term problem as a chronic illness persisting into adulthood. In addition, since the disease can present as an acute illness in adulthood, many adult patients with chronic illness from Still's Disease never show signs or symptoms of the disease in childhood, but both initially present with the disease in adulthood and may have persistence of all symptoms through some period of adulthood. The fever and systemic symptoms in these patients may go in remission, leaving arthritis symptoms. Conversely, all signs and symptoms, including arthritis and systemic signs and symptoms, may either remit completely during adulthood, present intermittently in a "flare" type pattern or, less commonly, continue indefinitely during adulthood.