Inflammatory Myopathy with Abundant Macrophages (IMAM)

Overview

Clinical Onset Ages: Childhood to 78 years Progression: Days to Weeks Children Most commonly asymptomatic Skin Rash: May be similar to dermatomyositis Edema Muscle Weakness: Proximal; Mild to Marked Myalgias Systemic features (25%) Arthralgias Raynaud's Lung infiltrate Treatment Corticosteroids Other immunomodulation Course Remission in some: After immunomodulating treatment Death: Occasional; Multiple organ failure may occur Laboratory Serum CK: Normal or Mildly high Aldolase: May be high with normal CK Serum autoantibodies: No consistent pattern Muscle pathology Histiocyte (Macrophage) infla

OMIM Entries

External Resources