多发性肌炎／皮肌炎合并间质性肺疾病的临床分析

目的探讨多发性肌炎／皮肌炎（PM／DM）合并间质性肺疾病（ILD）患者的临床表现、实验室检查、胸部影像学及肺功能的变化及意义。方法对住院的PM／DM并ILD患者7例（男性5例，女性2例，平均年龄55．7岁；5例为DM，2例为PM）均进行血清酶学和自身抗体测定、高分辨率CT（HRCT）及肺功能检查和皮肤肌肉活检。结果6例患者出现酶学增高，以肌酸激酶（CK）和乳酸脱氢酶（LDH）增高明显；4例患者抗核抗体（ANA）（＋），2例抗Jo-1抗体（＋）；皮肤肌肉活检病理诊断符合肌炎、皮肌炎改变。HRCT提示4例肺出现网点影及斑片阴影，3例出现双肺磨玻璃样病变和实变阴影。7例患者均出现限制性通气功能障碍，一氧化碳弥散量（DLCO）明显降低。6例患者使用糖皮质激素和免疫抑制剂治疗。5例患者治疗后病情稳定，1例患者死亡。结论HRCT可以及早发现PM／DM肺部病变。CK明显增高和抗Jo-1抗体阳性是诊断DM／PM并ILD的重要指标。患者可出现严重的弥散功能低下，应用激素合并免疫抑制剂治疗可取得较好疗效。

Clinical analysis of polymyosifis/dermatomyositis-associated interstitial lung disease

Objective To investigate the clinical situation, laboratory examination, chest radiology, pulmonary function in patients with polymyositis （ PM ） / dermatomyositis （ DM ） -associated interstitial lung disease （ILD）. Methods Seven patients with PM/DM-associated ILD were observed, 5 male and 2 female of them, mean age was 55.7 years, and 5 patients were DM, 2 patients were PM. Serum enzyme and autoantibody were determined, high resolution CT （HRCT） scanning, pulmonary function testing, skin and muscle biopsy were taken in all patients. Results The levels of creatine kinase （CK） and lactate dehydrogenase （LDH） increased significantly in six patients. Antinuclear antibody（ANA） was positive in four patients, and anti-Jo-1 antibody was positive in two patients. Skin and muscle biopsy indicated that pathology was in accord with PM or DM pathological characteristics in all patients; HRCT scanning showed that reticular and patching abnormalities in four patents, ground glass opacities and eonsoliclarion shadow in three patients. Pulmonary function testing showed that restrictive ventilatory functional disturbance, and diffusion capacity of the lung for carbon monoxide （DLCO） decreased significantly in all patients. After glucocorticoids and immtmodepressant were used in six patiems, five patient＇ s condition was stable, but one patient died. Conclusion HRCT may find early pulmonary changes in PM/DM. CK level increase markedly and positive anti-Jo- 1 antibody are important diagnostic indicators for PM/DM-associated ILD, and DLCO is decrease significantly in patients. Therapy with glucocorticoids and immunodepressant may make better curative effect.