Toyoshima M, Sato A, Chida K, Hayakawa H, Todate A, Imokawa S, Iwata M, Ohashi H, Shirai M, Yagi H
Department of Internal Medicine, Hamamatsu University School of Medicine, Japan.
Nihon Kyobu Shikkan Gakkai Zasshi. 1997 Mar;35(3):281-7.
We studied clinicopathological characteristics of interstitial pneumonia associated with amyopathic dermatomyositis. The subjects comprised two men and three women, and their mean age was 58.2 years. All subjects had cruptions specific for dermatomyositis, but had no signs of myositis. They all presented with acutely or subacutely developed coughing and dyspnea. Results of tests for anti-Jo-1 antibody were negative in all cases. Chest X-ray films showed infiltrations or streaky shadows, or both in the middle and lower lung fields. Analysis of bronchoalveolar lavage fluid revealed abnormally high percentages of lymphocytes and neutrophils. In one patients a specimen obtained by open lung biopsy showed homogeneous cell infiltrations in alveolar septa and regional alveolar damage. That patient was successfully treated with cyclosporin and corticosteroids in early phase of the disease. The other four patients received immunosuppressive agents after respiratory failure developed. All four died despite having received high-dose corticosteroid and immunosuppressive therapy. Examination of autopsy specimens showed diffuse alveolar damage.
我们研究了无肌病性皮肌炎相关间质性肺炎的临床病理特征。研究对象包括2名男性和3名女性，平均年龄为58.2岁。所有研究对象均有皮肌炎特有的皮疹，但无肌炎体征。他们均表现为急性或亚急性起病的咳嗽和呼吸困难。所有病例抗Jo-1抗体检测结果均为阴性。胸部X线片显示中、下肺野有浸润影或条索状阴影，或两者皆有。支气管肺泡灌洗 fluid分析显示淋巴细胞和中性粒细胞百分比异常升高。1例患者经开胸肺活检获取的标本显示肺泡间隔有均匀的细胞浸润及局部肺泡损伤。该患者在疾病早期成功接受了环孢素和皮质类固醇治疗。另外4例患者在出现呼吸衰竭后接受了免疫抑制剂治疗。尽管接受了大剂量皮质类固醇和免疫抑制治疗，4例患者均死亡。尸检标本检查显示弥漫性肺泡损伤。
