Clinical Rheumatology

, Volume 33, Issue 3, pp 305–314

Life-threatening complications of adult-onset Still’s disease

Review Article

DOI: 10.1007/s10067-014-2487-4

Cite this article as:
Efthimiou, P., Kadavath, S. & Mehta, B. Clin Rheumatol (2014) 33: 305. doi:10.1007/s10067-014-2487-4

Abstract

Adult-onset Still’s Disease (AOSD) since its description in 1971 has proven to be a very complex and challenging disease entity. This rare auto-inflammatory disease is classically described by the “Still’s triad” of fever, rash, and arthritis, although the atypical cases frequently outnumber the typical ones. The exact pathogenesis and etiologic factors responsible for the clinical features remain largely obscure, despite recent suggestive cytokine biology findings. Diagnosis is made on clinical grounds, following the exclusion of mimickers of infectious, autoimmune or neoplastic etiology, with the additional consideration of non-specific laboratory abnormalities such as peripheral leukocytosis and elevation of serum ferritin and other acute phase reactants. The disease manifestations are protean and can include diverse complications, affecting multiple organ systems. Moreover, the severity of the organ involvement can vary considerably, representing a wide spectrum from the self-limited to severe. The mainstay of therapy has evolved from the traditional use of corticosteroids and oral immunosupressants to the newer targeted treatments with biologic agents. The scope of this review is to alert the clinician to the existence of life-threatening AOSD complications, namely the macrophage activation syndrome, disseminated intravascular coagulopathy, thrombotic thrombocytopenic purpura, diffuse alveolar hemorrhage, and pulmonary arterial hypertension. Such knowledge may lead in earlier recognition, prompt treatment, and, ideally, improved patient outcomes.

Keywords

Adult-onset still’s disease Complications Etiology Macrophage activation syndrome (MAS) Pathogenesis Treatment 

Copyright information

© Clinical Rheumatology 2014

Authors and Affiliations

  • Petros Efthimiou
    • 1
    • 2
  • Sabeeda Kadavath
    • 1
  • Bella Mehta
    • 3
  1. 1.Rheumatology DivisionLincoln Medical and Mental Health CenterNew YorkUSA
  2. 2.Weill Cornell Medical CollegeNew YorkUSA
  3. 3.Westchester Medical CenterNew YorkUSA

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