Asanuma Y, Yamada H, Matsuda T, Hoshi K, Ichikawa Y, Mizushima Y, Niimi H
Department of Internal Medicine and Laboratory Medicine, Saint Marianna University School of Medicine, Kawasaki-city, Kanagawa.
Ryumachi. 1997 Oct;37(5):719-26.
Interstitial pneumonia complicated with dermatomyositis sometimes shows a resistance to high dose steroid therapy and a fatal course particularly in patients without showing the elevation of creatine kinase. We experienced a 48 year old woman who developed heliotrope rash, Gottron's sign, multiple cutaneous ulcers, and dyspnea on exertion. These symptoms were resistant to low dose steroid therapy. Serum levels of creatine kinase were normal. Anti-nuclear antibodies and anti-Jo-1 antibody were negative. High resolution CT scan of the chest showed areas of multiple air space consolidation and subpleural linear shadows. Lung biopsy performed under video-assist thoracosurgery revealed diffuse alveolitis with scattered lymphoid folicules and mild accumulation of macrophages in the alveolar spaces. There were no honey-combing. These features were compatible with "non-specific interstitial penumonia" proposed by Katzenstein, 1995. The patient was treated with 10 micrograms lipo-PGE1, PGE1 incorporated in lipid microspheres, and 300 mg pentoxifylline, which resulted in a dramatic improvement of both interstitial pneumonia and cutaneous ulcers. The present case suggested a novel strategy for the treatment of interstitial pneumonia.
