Life Science Encyclopedia

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 (AOSD) described by another english physician, Bywaters on 1971 (the same author is famous for the crush syndrome).

There are several theories about the cause of Still's disease. It has been suggested it may be caused by a microbacterial infection. However, the cause of Still's disease remains unknown. The pathogenesis of the disease is more secure to be autoimmune.

Symptoms

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
• Severe joint pain and joint damage

Diagnosis

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. A new important marker of AOSD is the glycosylated ferritin: the percentage of this form of the protein is often below the 50% of the total during the flare of the disease and often during the remission phases.

Prognosis

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.