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This retrospective single-center cohort study included consecutive adult patients with dermatomyositis, polymyositis and anti-synthetase syndrome with severe manifestations (dysphagia, bedridden muscle weakness, dyspnea secondary to interstitial lung disease, vasculitis or cutaneous rash >30% of body surface area) from 2000 to 2017. Exclusion criteria: overlap syndromes, cancer induced myositis, followup <6 months not caused by death. Three main outcomes were assessed: complete clinical response [1], glucocorticoid withdrawal and mortality. Three different induction therapies were compared: oral glucocortidoid (group 1), pulse methylprednisolone (group 2) and pulse methylprednisolone plus intravenous immunoglobulin (IVIG) (group 3). Cox proportional hazards modeling and Kaplan-Meier analysis were applied to determine the differences between treatment groups.

blucher medical proceedings

INDUCTION THERAPY WITH ORAL GLUCOCORTICOID, PULSE METHYLPREDNISOLONE OR PULSE METHYLPREDNISOLONE PLUS INTRAVENOUS IMMUNOGLOBULIN IN PATIENTS WITH SEVERE DERMATOMYOSITIS, POLYMYOSITIS AND ANTI-SYNTHETASE SYNDROME