7. Life-threatening adult-onset Still’s disease

摘要

IntroductionAdult-onset Still’s disease (AOSD) is a rare inflammatory disorder of unknown aetiology with systemic involvement. The presentation and severity is highly variable in affected individuals and requires a clinical diagnosis, commonly using the Yamaguchi classification criteria. The majority of patients with AOSD develop non-specific symptoms associated with a systemic inflammatory response including fever, rash and arthralgia; however, a few patients with AOSD develop life-threatening complications. Here, we describe a case of newly diagnosed AOSD who rapidly deteriorated with myo-pericarditis, cardiogenic shock and multi-organ failure requiring ECMO, despite treatment with high dose oral corticosteroids.