Adult onset Still disease
Alerts and Notices
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
Look For
Subscription Required
Diagnostic Pearls
Subscription Required
Differential Diagnosis & Pitfalls
Infections: Immune mediated:Best Tests
Subscription Required
Management Pearls
Subscription Required
Therapy
Subscription Required
References
Subscription Required
Last Reviewed:02/14/2019
Last Updated:06/24/2020
Last Updated:06/24/2020