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.
KEY INDEXING TERMS
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Article info
Publication history
Accepted:
September 1,
2006
Received:
August 1,
2006
Identification
Copyright
© 2007 Southern Society for Clinical Investigation. Published by Elsevier Inc. All rights reserved.