Granulomatous Myopathies

Overview

Clinical features Weakness: Proximal; Dysphagia Muscle hypertrophy: Occasional Progression: Slow; Occasionally acute with pain Post-menopausal females Some patients with progressive flexion contractures of arms Treatment Corticosteroids: Benefit General: 40% to 80% More likely: Associated systemic sarcoidosis Less likely: Isolated granulomatous myopathy; IBM-like clinical syndrome Laboratory Serum CK: Normal or High Aldolase: ? More often elevated than CK Pathology Granulomas Non-caseating Giant cells Some biopsies Multinucleated CHIT1 staining 147 Location in muscle Endomysium or Perimysial c

OMIM Entries

Related Conditions

External Resources