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Table 1 Characteristics of life-threatening AOSD

From: Diagnostic and management of life-threatening Adult-Onset Still Disease: a French nationwide multicenter study and systematic literature review

  n (%)
Characteristics of patients
 Age, years (median) 36 (18–64)
 Male 12 (60%)
 First episode 18 (90%)
AOSD features
 Fever 20 (100%)
 Rash 13 (65%)
 Sore throat 13 (65%)
 Arthromyalgia 17 (85%)
 Arthritis 3 (15%)
 Hepatosplenomegaly 13 (65%)
Organ manifestations
 Respiratory
  Acute respiratory failure 15 (75%)
  Lung infiltrate 12 (60%)
  Pleural effusion 12 (60%)
 Cardiocirculatory
  Shock 10 (50%)
  Myocarditis 8 (40)
  Pericarditis 10 (50%)
  Tamponade 3 (16%)
 Hematologic
  Disseminated intravascular coagulation 10 (50%)
  Hemophagocytosis 8 (40%)
  Thrombotic microangiopathy 1 (5%)
 Other
  Acute kidney injury 7 (35%)
  Neurologic dysfunction 5 (25%)
  Severe hepatitis 3 (15%)
  Multiple organ failure 8 (40%)
  Death 2 (10%)