Skip Navigation LinksHome > March 2007 - Volume 333 - Issue 3 > A Case of Hypomyopathic Dermatomyositis Associated with Rapi...
American Journal of the Medical Sciences:
doi: 10.1097/MAJ.0b013e318031b122
Case Reports

A Case of Hypomyopathic Dermatomyositis Associated with Rapid Progressive Interstitial Pneumonia Resistant to Multi-immunosuppressive Therapy.

Tsuda, Tokutaro MD; Asanuma, Yu MD, PhD; Koyama, Shinichiro MD, PhD; Kawabata, Yoshinori MD, PhD; Moriguchi, Masato MD, PhD

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Abstract

The prognosis of interstitial pneumonia in patients with dermatomyositis is thought to depend on the histologic type and response to therapy. We report a case of hypomyopathic dermatomyositis with rapid development of a fatal interstitial pneumonia. Although the histology of lung biopsy indicated minimal alveolitis, the patient died despite multi-immunosuppressive therapy. The autopsy revealed acute and organizing diffuse alveolar damage. In this case, the lung biopsy taken before the acute deterioration did not predict the patient's poor prognosis. Interstitial pneumonia in patients with amyopathic or hypomyopathic dermatomyositis would develop rapidly progressive and refractory. We need larger and better prospective studies to determine whether early and intensive immunosuppressive therapy improves the prognosis of interstitial pneumonia in patients with amyopathic or hypomyopathic dermatomyositis.

© Copyright 2007 Southern Society for Clinical Investigation

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