Adult Onset Still’s Disease (AOSD)

Happy Monday!

Thank you, Tim and Polly, for presenting the case of a young woman who presented with fevers, rash, and polyarthralgias, and was found to have elevated inflammatory markers and ferritin. She was diagnosed with likely Adult Onset Still’s Disease and started on steroids.

Love,
SamMy

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TOP PEARLS

  • When evaluating a patient with polyarthralgias and rash, time course can be helpful in terms of the various etiologies (infection vs autoimmune vs malignancy). Symptoms for 1-2 weeks may point towards viral, whereas longer may suggest autoimmune.
  • Adult Onset Still’s Disease is a diagnosis of exclusion, and hence often takes months to be diagnosed.
  • Use the Yamaguchi criteria to diagnose Still’s Disease. The 4 major criteria are: fever, arthritis, rash, and leukocytosis.

 

MORE ON ADULT ONSET STILL’S DISEASE (AOSD)

  • It is a diagnosis of exclusion!
  • Yamaguchi Criteria (4 major criteria):
    • Fever of at least 39ºC (102.2ºF) lasting at least one week
    • Arthralgias or arthritis lasting two weeks or longer
    • A nonpruritic macular or maculopapular skin rash that is salmon-colored in appearance and usually found over the trunk or extremities during febrile episodes
    • Leukocytosis (10,000/microL or greater), with at least 80 percent granulocytes
    • Minor criteria include a sore throat, LAD/splenomegaly, liver dysfunction, and negative RF and ANA
  • The characteristic rash in Still’s is characteristically nonpruritic, evanescent (correlates with time of fever), and salmon-colored! Distribution is often on the chest.
    • However, there are case reports of urticarial rashes as the presenting manifestation of Still’s (PMID 11055830, PMID 17241583)
  • Treatment:
    • ASA or NSAIDs as initial treatment (response rate is 20-25%, and are 1st line therapy for mild disease)
    • Steroids (95% efficacy; for moderate to severe disease)
    • DMARDS (eg anakinra, an IL1 receptor antagonist, most commonly used in RA)
  • Prognosis:
    • Predictors of unfavorable outcome/chronic disease include development of erosive polyarthritis in the initial presentation, and involvement of shoulders or hips. Systemic symptoms are generally not predictive of poorer outcomes.


POLYARTHRITIS

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