Bronchiolitis obliterans organizing pneumonia as the first manifestation of polymyositis.

Bronchiolitis obliterans organizing pneumonia (BOOP) preceding polymyositis is rare. In this report, a 51-year-old patient with fever, nonproductive cough, and dyspnea had bilateral basal interstitial infiltrates on chest roentgenogram. Open lung biopsy was consistent with BOOP. Prednisone therapy led to improvement, but 8 weeks later, fever, cough, and weakness of the arms and legs developed because the patient had not been compliant with the prednisone regimen. The creatine kinase (CK), the macrophage inflammatory protein (MIP-1), and the tumor necrosis factor (TNF-alpha) were elevated. Anti-Jo-1 antibody was not present. Quadriceps femoris muscle biopsy was compatible with polymyositis. After a second course of corticosteroid therapy, the patient became afebrile, the dyspnea resolved, the pulmonary infiltrates decreased, and the muscle strength improved. The serum CK, MIP-1, and TNF-alpha levels declined significantly. This is only the second reported case of BOOP preceding polymyositis. Patients with idiopathic BOOP should have follow-up for the possible development of connective tissue disorders including polymyositis.