Nanke Y, Tateisi M, Yamagata H, Hara M, Kamatani N
Department of Internal Medicine, National Murayama Hospital, Tokyo.
Ryumachi. 2000 Aug;40(4):705-10.
We report here a case of interstitional pneumonia (IP) associated with amyopathic dermatomyositis (DM). In August, 1998, a 53-year-old Japanese man was admitted to our hospital because of fever, polyarthritis and erythematous heliotrope eruption and Gottron's sign without any symptom of myositis. Serum CK level and EMG were normal. Jo-1 antibody was negative. Chest computed tomography (CT) revealed IP at both lung base areas. The patient was diagnosed as amyopathic DM with IP. When IP rapidly progressed, methylprednisolone pulse therapy and oral high dose prednisolone were not effective. High-dose intravenous cyclophosphamide seemed to be transiently effective, but oral cyclosporine A was not effective. The patient was died of respiratory failure in October 1998. During the course, we measured serum levels of LDH, sIL-2 R, s-ICAM-1 and KL-6, KL-6 could be a sensitive parameter of IP activity.
我们在此报告一例与无肌病性皮肌炎（DM）相关的间质性肺炎（IP）病例。1998年8月，一名53岁的日本男性因发热、多关节炎、红斑性向阳疹和Gottron征入院，无任何肌炎症状。血清肌酸激酶（CK）水平和肌电图正常。Jo - 1抗体阴性。胸部计算机断层扫描（CT）显示双肺底部区域有间质性肺炎。该患者被诊断为伴有间质性肺炎的无肌病性皮肌炎。当间质性肺炎迅速进展时，甲泼尼龙冲击疗法和口服大剂量泼尼松无效。大剂量静脉注射环磷酰胺似乎有短暂疗效，但口服环孢素A无效。该患者于1998年10月死于呼吸衰竭。在此过程中，我们检测了血清乳酸脱氢酶（LDH）、可溶性白细胞介素 - 2受体（sIL - 2R）、可溶性细胞间黏附分子 - 1（s - ICAM - 1）和KL - 6的水平，KL - 6可能是间质性肺炎活动的一个敏感指标。
