Adult-onset Still’s disease: what does the intensivist need to know?

Authors

  • Mathieu Gerfaud-Valentin Service de Médecine Interne, Hôpital de la Croix Rousse, Hospices Civils de Lyon, Université Claude Bernard Lyon 1, Lyon, France
  • Jean-Christophe Richard Service de Médecine Interne, Hôpital de la Croix Rousse, Hospices Civils de Lyon Université Claude Bernard Lyon 1, Lyon, France. Service de Médecine Intensive - Réanimation, Hôpital de la Croix Rousse, Hospices Civils de Lyon, Université Claude Bernard Lyon 1, Lyon, France.
  • Maxime Fauter Service de Médecine Interne, Hôpital de la Croix Rousse, Hospices Civils de Lyon, Université Claude Bernard Lyon 1, Lyon, France
  • Pascal Sève Service de Médecine Interne, Hôpital de la Croix Rousse, Hospices Civils de Lyon, Université Claude Bernard Lyon 1, Lyon, France Faculté de Médecine et de Maïeutique Lyon Sud - Charles Mérieux
  • Yvan Jamilloux Service de Médecine Interne, Hôpital de la Croix Rousse, Hospices Civils de Lyon, Université Claude Bernard Lyon 1, Lyon, France Faculté de Médecine et de Maïeutique Lyon Sud - Charles Mérieux Centre International de Recherche en Infectiologie (CIRI), Inserm U1111

DOI:

https://doi.org/10.37051/mir-00034

Keywords:

Adult-onset Still's disease, hemophagocytic lymphohistiocytosis, anakinra

Abstract

Adult-onset Still’s disease (AOSD) is a rare multigenic autoinflammatory disorder of which two phenotypes may be distinguished: a chronic articular pattern and a systemic (mono- or polycyclic) pattern. In the latter, life-threatening complications may occur in a third of patients, often at onset or during the first year of the disease course. The most common one are hemophagocytic lymphohistiocytosis, disseminated intravascular coagulation, myocarditis, acute liver failure, and multiple organ failure. This review emphasizes the diagnostic workup and specific treatments of these severe patterns of AOSD in the light of up to date data from the medical literature.

Published

2020-10-12

How to Cite

Gerfaud-Valentin, M., Richard, J.-C., Fauter, M., Sève, P., & Jamilloux, Y. (2020). Adult-onset Still’s disease: what does the intensivist need to know?. Médecine Intensive Réanimation, 29(3), 203-216. https://doi.org/10.37051/mir-00034