Abstract

3 new cases of dermatomyositis associated with diffuse interstitial pulmonary fibrosis and, more exceptionally, with Sjögren's syndrome are reported. The pulmonary fibrosis observed in patients with dermatomyositis differs from that found in other connective tissue diseases in that it follows a more acute course and may respond to corticosteroids. Thus, in 2 of these patients treated with corticosteroids (combined in 1 case with cyclophosphamide) the high percentage of lymphocytes and polymorphonuclears in the broncho-alveolar lavage fluid, which reflects alveolitis activity, was reduced and the pulmonary fibrosis was cured in one patient and stabilized in the other.

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