We describe five cases of polymyositis/dermatomyositis with interstitial lung disease, occurring in women, with a mean follow up of 28 months (15 to 51). One remained without respiratory symptoms, two had a chronic and relapsing course, and two an acute course, leading to death with respiratory failure in one. Anti-Jo1 antibody was found in two patients, without relation to the gravity of the respiratory illness. The two others with a severe course had anticardiolip antibodies, and one cutaneous necrosis of the fingers. Cancer-associated dermatopolymyositis was never seen at diagnosis, but a lymphoma of the brain was diagnosed after two years in one patient. Combination of corticosteroids with another immunomodulator treatment was necessary for all, because of frequent relapses. Methotrexate was effective in one case for the muscular and the respiratory signs, plasma exchanges with azathioprine in two cases, and intravenous immunoglobulin in one patient with acute lung injury.