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[Adult onset Still's disease]
1Service d'immunologie et allergologie, Département de médecine interne, HUG, 1211 Geneve 14. camillo.ribi@hcuge.ch
Revue Medicale Suisse
|June 19, 2008
Summary
Adult onset Still's disease (AOSD) is a rare autoimmune condition. Early diagnosis and treatment with corticosteroids or methotrexate are key, with cytokine blockade offering new hope for refractory cases.
Area of Science:
- Rheumatology
- Immunology
- Internal Medicine
Context:
- Adult onset Still's disease (AOSD) is a rare systemic inflammatory disorder.
- Primarily affects young adults, presenting with fever, arthritis, rash, and organomegaly.
- Diagnosis is challenging due to non-specific features and is often by exclusion.
Purpose:
- To summarize the key clinical and biological characteristics of AOSD.
- To highlight diagnostic challenges and current treatment strategies.
- To discuss emerging therapeutic targets like cytokine blockade.
Summary:
- AOSD presents with fever, arthritis, rash, sore throat, lymphadenopathy, and splenomegaly.
- Biologically, it involves neutrophilic leukocytosis, high ferritin, and elevated liver enzymes.
- Current treatments include corticosteroids and methotrexate, with cytokine inhibitors showing promise.
Impact:
- Improved understanding of AOSD pathogenesis and clinical manifestations.
- Guidance for clinicians in diagnosing and managing this rare condition.
- Highlights the potential of targeted therapies for patients unresponsive to conventional treatments.
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