Adult onset Still’s disease as the cause of fever of unknown etiology
Journal Title: Postępy Nauk Medycznych - Year 2012, Vol 25, Issue 2
Abstract
Adult-onset Still’s disease (AOSD) is a systemic inflammatory disease of unknown etiology to be differentiate with sepsis in first period of active process. AOSD is characterized by a combination of several clinical symptoms and laboratory disturbances but predominantly high spiking fever, arthritis, maculopapular salmon rash and leukocytosis can appeared. However, systemic manifestations as hepatomegaly, splenomegaly, pneumonia, myocarditis and pericarditis may often occur.<br>The diagnosis of AOSD is often problematic and require clinical experience. Despite of various pro-inflammatory cytokine profiles (interleukin-1, interleukin-6, interleukin-18, TNF-α, interferon-γ) only clinical manifestations can differentiate AOSD from others active diseases and particular from systemic sepsis. It seems that clinical rheumatologist with good internal medicine backgroud is the best prepared consulting – specialist who should take care of these patients for a long time. There is no single diagnostic test to confirm AOSD also classification – diagnostic criteria and activity indexes did not be yet established. The treatment oriented to control and symptoms and signs.<br>There are no randomized controlled clinical trials in AOSD so scientific evidence for therapy is poor. Recommendations derived from retrospective analysis and several case reports. The first choice of treatment are high doses of corticosteroids and methotrexate. Biologic agents are second line therapy and their application require further studies and evaluations.
Authors and Affiliations
Piotr Leszczyński, Katarzyna Pawlak-Buś, Tomasz Szafrański
Keywords
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