Adult-onset Still disease
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Synopsis
AOSD is characterized by spiking fevers that typically peak in the late afternoon or early evening, a pink or salmon-colored evanescent rash, polyarthralgia, hepatosplenomegaly, lymphadenopathy, and sore throat. Fever, rash, and arthralgias occur in most patients. Arthralgias and arthritis typically affect the knees, ankles, and wrists.
There are 3 different patterns in the clinical evolution of AOSD. The monocyclic pattern is self-limited and characterized by one flare of variable duration followed by complete remission. The polycyclic pattern is marked by 2 or more episodes with intervening symptom-free periods. The chronic articular pattern is characterized by severe joint manifestations that lead to joint-space narrowing and destruction. AOSD can also have serious systemic complications including serositis, chronic arthropathy, and macrophage activation syndrome. Late carpal ankylosis occurs in approximately 25% of patients and represents a distinct clinical feature differentiating AOSD from rheumatoid arthritis.
Codes
ICD10CM:M06.1 – Adult-onset Still's disease
SNOMEDCT:
239920006 – Adult onset Still's disease
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Last Reviewed: 02/15/2019
Last Updated: 03/01/2019
Last Updated: 03/01/2019
Adult-onset Still disease
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