A Pediatric Case of Granulomatous Myositis and Response to Treatment

Idiopathic inflammatory myopathy encompasses a group of acquired, heterogeneous, systemic diseases of the skeletal muscle, including adult polymyositis, adult dermatomyositis, juvenile dermatomyositis, juvenile polymyositis, inclusion body myositis, and necrotizing myopathy, all resulting in muscle weakness. Granulomatous myositis (GM) is a rare myopathy disorder histologically characterized by the development of endomyseal and/or perimyseal granulomas in striated muscle. GM is often associated with sarcoidosis. GM has also been associated with myasthenia gravis, inflammatory bowel disease, thymoma, and malignancy. We are reporting a rare case of a 13-year-old girl with GM without associated disease that was refractory to multiple medications, and responded well to rituximab.