Definition: Still’s disease is a form of arthritis. Stills disease is characterized by high spiking fevers and evanescent (transient) salmon-colored rash (view pictures of the Still’s rash). Still’s disease was first described in children, but it is now known to occur, much less commonly, in adults (it is referred to as Adult-onset Still’s disease, AOSD). Cause: There are 2 concepts for the cause: 1st is assumed to be that Still’s disease is due to infection with a microbe. Another concept is that Still’s disease is a hypersensitive or autoimmune disorder. Yet the cause of Still’s disease is still not known. Pathophysiology: The cause of adult-onset Still's disease is not known, but it presumably involves interleukin-1 (IL-1), since drugs that block the action of IL-1ß are effective in treatment. Interleukin-18 is expressed at high levels
Symptoms: Still’s disease usually present with systemic (body wide) symptoms. Extreme fatigue can have high fevers that rise to 104 degrees F (41 degrees C) or even higher and rapidly return to normal levels or below. A faint salmon-colored skin rash. There is 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). Treatment: Symptoms are often controlled with anti-inflammatory drugs, such as aspirin or other non-steroid drugs (NSAIDs). Cortisone medications (steroids), such as prednisone, are used to treat more severe features of the illness. Patients with persistent illness, medications that affect the inflammatory aspects of the immune system are used. Medications now being used are analogous to the classic “second-line” therapies used for patients with Rheumatoid Arthritis. These include Gold, Hydroxychloroquine (PLAQUENIL), Penicillamine, Azathioprine (IMURAN), Methotrexate(RHEUMATREX), and Cyclophosphamide.
Statistics: The approximate Adult Stills Disease Incidence in Western Europe, Ireland was found to be 1468. Adult-onset Still's Disease is rare and has been described all over the world. The number of new cases of AOSD per year is estimated to be 0.16 new cases per 100,000 populations. Prevalence is estimated at 1.5 cases per 100,000-1,000,000 population.