Adult onset Still's disease (juvenile rheumatoid arthritis with septic appearance) is rare, leading to clinical signs similar to those seen in bacterial sepsis, lymphomas, rheumatological, or systemic autoimmune diseases. The disease can present with a fever of unknown origin, and can cause difficulties in the diagnosis. It is based upon, partly, the exclusion of other diseases and on diagnostic criteria. Its characteristic feature is the rise of acute phase proteins. Exanthemata are temporary. The basis of treatment is immunosuppression, however relapses can occur.
The aim of the authors was to evaluate on the most characteristic clinical signs and laboratorical data of their patients, and to examine the revealing parameters of the course of the disease.
Retrospective epidemiological survey of the data obtained from 18 patients.
The characteristic signs of the disease were, fever, sore throat, arthritis, joint pain, exanthemata, hepato-splenomegaly, lymphadenomegaly, pleurisy. The typical laboratorical data were: elevated CRP, low PCT, negative Waaler-Rose and ANA test, low serum iron level, leukocytosis, thrombocytosis, elevated alkalic phosphatase activity, high LDH, positive bone scintigraphy. The fever was steroid dependent. Generally, the illness was recognised after 2-3 months, and relapses were frequent.
Still's disease has an important role in the differential diagnosis of fever of unknown origin. The diagnosis is based upon the evaluation of clinical signs and laboratorical data together. Prolonged immunosuppressive therapy is required.