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Results 11/116 DMJ (9.3%) (8 girls and 3 boys, median age at diagnosis : 9.0± 3.1 years) were admitted in ICU for digestive involvement (2 with digestive perforation after pulse corticosteroids) (4 patients), bradycardia (1 patient), cardiac arrest (1 patient), hypoxemic pneumonia (1 patient), PRES syndrome due to cyclosporine (1 patient), thrombotic microangiopathy (TMA) (2 patients) and anaphylactic shock due to Rituximab (1 patient). The incidence of some clinical and biological manifestations differed from severe patients to patients with mild JDM: hyponatremia (9), hypoalbuminemia (9), generalized edema (6), anemia (hemoglobin value <8 g/dL) (8), abdominal pain (7), thrombocytopenia (platelet count : 100-150 x 10/L )(7). The patients were treated by corticosteroids (11, comprising 5 with pulse), intravenous immunoglobulins (7), plasmapheresis (7), Rituximab (4) and cyclophosphamide (2). One patient died in ICU from pneumocystosis ; 5 are currently in complete remission and 5 in partial remission with a mean follow-up duration of 4,7 years.

Clinical characteristics, management and outcomes in patients with juvenile dermatomyositis requiring admission in pediatric intensive care unit