Among 5,885 sarcoidosis patients, 21 had symptomatic myopathy. Eight carried diagnosis of sarcoidosis 5.5 years (median) prior to myopathy onset. Eleven patients had SM. The remaining had non-sarcoid myopathies (5 IBM, 1 immune-mediated necrotizing myopathy, 1 nonspecific myositis, 2 nonspecific myopathy and 1 steroid myopathy). Estimated frequency of IBM is 85 per 100,000 sarcoidosis patients. The following features were associated with non-sarcoid myopathies (p<0.05): 1) predominant finger flexor and quadriceps weakness, 2) modified Rankin scale (mRS) >2 at time of diagnosis, 3) creatine kinase > 500 U/L, and 4) absence of intramuscular granulomas. Sarcoplasmic MxA expression was observed in scattered myofibers in 3 patients, 2 of whom tested for dermatomyositis-specific autoantibodies and were negative. Immunosuppressive therapy led to improvement in mRS ≥ 1 in 5/10 SM, none of the 5 IBM, and 3/3 remaining patients with non-sarcoid myopathies.