Abstract
A 61-year-old female developed a fever and productive cough in April 2005. Chest radiography showed a reticular shadow, and she was admitted to a local hospital. Chest CT scanning revealed streaky shadows and patchy consolidations surrounded by ground-glass opacities predominantly on the dorsal side of bilateral lungs. Although steroid pulse therapy and several antibiotics were administered, she was transferred to our hospital due to rapid progression of respiratory failure. Laboratory findings showed the following; LDH 726 U/I; CK 490 U/I; and KL-6 824 U/ml. Autoantibodies such as anti-Jo-1 antibody were all negative. Eruptions consistent with heliotrope rash and Gottron’s sign were observed, but muscle symptoms were mild, and CK levels were slightly increased. She was diagnosed as having rapidly progressive interstitial pneumonia accompanied by dermatomyositis without muscle symptoms. Under mechanical ventilation, she received steroid pulse therapy, cyclosporine-A, and cyclophosphamide pulse therapy. On the 10th hospital day, she was liberated from artificial ventilation. Follow up chest CT scanning showed a marked improvement of interstitial pneumonia. Cases of progressive interstitial pneumonia complicated with dermatomyositis that are negative for anti-Jo-1 antibody and show a low ratio of CK/LDH are resistant to various treatments. The 3-drug combination therapy consisted of corticosteroids, cyclosporine-A and cyclophosphamide may be effective for rapidly progressive interstitial pneumonia complicated with dermatomyositis.
