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Adult-Onset Still’s Disease

  • Petros Efthimiou
  • Sujani Yadlapati
Chapter

Abstract

Adult-onset Still’s disease (AOSD) is a rare systemic, autoinflammatory disorder that often presents in adolescence and early adulthood with fever, rash, and polyarthritis. There are significant genetic and clinical similarities with systemic juvenile idiopathic arthritis (sJIA) with a different chronological disease onset. The disease can have many protean characteristics leading to delays in diagnosis. Treatment includes corticosteroids; traditional immunomodulators, such as methotrexate; and targeted biologic treatments that include IL-1 and IL-6 inhibitors.

Keywords

Adult-onset Still’s disease Pathogenesis Etiology Treatment Macrophage activation syndrome (MAS) IL-1 inhibitors 

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Copyright information

© Springer Nature Switzerland AG 2019

Authors and Affiliations

  1. 1.New York University School of MedicineNew YorkUSA
  2. 2.NYU/Langone HealthNew YorkUSA
  3. 3.Department of Internal MedicineAlbert Einstein Medical CenterPhiladelphiaUSA

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