We describe the case of a patient with polymyositis and anti-Jo-1 antibodies who experienced a relapse of his inflammatory muscle disease after 7 years of clinical remission. Our review of the literature shows that remission is achieved in 25-68% of patients treated with high dose corticosteroids as part of their initial therapy. Relapse rates after complete remission vary from 6 to 43% in the few studies where this information is available. Late relapses after initial remission appear to be unusual, but may be more frequent in patients like this one with antisynthetase syndrome.