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). 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). Diagnosis: Diagnosis for Still’s disease develops markedly elevated white blood cell counts, as if they have a serious infection but none is found. Low red blood counts (anemia) and elevated blood tests for inflammation (such as sedimentation rates) are common. However, the classic blood tests for Rheumatoid Arthritis (rheumatoid factor) and systemic Lupus erythematosus (antinuclear antibodies, ANA) are usually negative.
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. These include Gold, Hydroxychloroquine (PLAQUENIL), Penicillamine, Azathioprine (IMURAN), Methotrexate(RHEUMATREX), and Cyclophosphamide. There is a new class of drugs called biologics that are very promising in treating Still’s. Enbrel, Remicade, Kineret and several others are available and are being used as a first line therapy in treating still’s meaning you don’t have to take Methotrexate
Statistics: The approximate Adult Stills Disease Incidence in Western Europe, Belgium was found to be 56,728. 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.