[Interstitial fibrosing pneumopathy and dermatomyositis. Pulmonary histology and prognosis]

The authors describe a case of interstitial lung disease associated with dermatomyositis. Alveolar-interstitial shadowing on the initial chest X-ray, lymphocytosis on the broncho-alveolar lavage fluid, absence of anti-JO1 antibodies and presence of intra-alveolar buds on the open lung biopsy were observed. Our patient died of acute respiratory failure, 34 months after symptoms onset. Intra-alveolar buds found at an early stage of dermatomyositis interstitial lung disease, were associated with minimal interstitial cellularity, markedly interstitial fibrosis, poor prognosis and poor response to steroid therapy. In contrary, bronchiolitis obliterans organizing pneumonia noted at an early stage of interstitial lung disease associated with both dermatomyositis and polymyositis, seems to have better prognosis and response to corticosteroid, especially if associated with polymyositis.