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A patient with amyopathic dermatomyositis associated with fatal rapidly progressive interstitial pneumonitis resistant to therapy is described. Pathologic examination of a transbronchial lung biopsy specimen showed diffuse alveolar damage and nonspecific interstitial pneumonia-organizing pneumonia-like findings. Bronchoalveolar lavage fluid contained many CD8+ lymphocytes, consid-ered to be cytotoxic T cells. Analysis of bronchoalveolar lavage fluid in this case may provide prognostically and pathogenetically important information. Key words: amyopathic dermatomyositis, interstitial pneu-monitis, bronchoalveolar lavage, cytotoxic T cell A subgroup of patients with dermatomyositis (DM) develop rapidly fatal progressive interstitial pneu-monitis that is unresponsive to corticosteroid ther-apy. This group of patients tends to complain of mild muscle symptoms and have normal serum cre-atine kinase and blood lymphocyte levels and neg-ative tests for anti-Jo-1 antibody [1]. This type of DM is designated amyopathic DM. Here, we describe a patient with amyopathic DM who devel-oped rapidly progressive interstitial pneumonitis and discuss differential diagnosis, treatment, patho-logic features, and findings in bronchoalveolar lavage. Case Report A 50-year-old woman with DM who developed interstitial pneumonitis with acute respiratory fail-ure was transferred to the Emergency and Critica