Adult-onset Still’s disease: what does the intensivist need to know?
Keywords:Adult-onset Still's disease, hemophagocytic lymphohistiocytosis, anakinra
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.