Table 1 Key features of catastrophic adult-onset Still disease

Adult-onset Still disease

 Yamaguchi and/or Fautrel criteria

 Usually new-onset disease

AND ≥ 1 organ failure requiring ICU management

 Acute respiratory failure, caused by

  Lung infiltrate

  Pleural effusion

  Cardio-circulatory failure

 Cardio-circulatory failure, caused by

  Non-cardiogenic shock

  Myocarditis

  Tamponade

 Haematologic disorder, caused by

  Disseminated intravascular coagulation

  Haemophagocytosis

  Thrombotic microangiopathy

AND no alternative cause

 No infection

 No drug reaction

 No malignancy