A clinical analysis of dermatomyositis with interstitial lung disease in 20 patients

目的 探讨皮肌炎(DM)合并间质性肺疾病(ILD)的临床特点.方法 回顾性分析20例DM合并ILD患者的临床资料.结果 169例DM中合并ILD者20例,发病率为11.8％,4例无呼吸系统症状.11例血清肌酸激酶升高,4例抗Jo-1抗体阳性.胸部高分辨率CT主要表现为磨玻璃影、小叶间隔增厚和不规则线状、网格影.肺功能以限制性通气功能障碍和弥散功能下降为主,15例患者存在不同程度的低氧血症或呼吸衰竭.经糖皮质激素和(或)免疫抑制剂治疗后,14例患者病情明显好转,2例无缓解,4例死亡.结论 DM合并ILD发生率高,病死率高,临床易漏诊,应提高对本病的认识,早诊断早治疗,最大程度改善患者的预后.Objective To investigate the clinical features of the patients with dermatomyositis (DM)complicated with interstitial lung disease(ILD).Methods The clinical manifestations and the laboratory data of the 20 patients with DM complicated with ILD were retrospectively analyzed.Results Among 169 DM patients,20(11.8％)were founded with ILD and 4 had no complaint of respiratory system.The ratio of male to female was 1∶2.33.The level of serum creatine kinase(CK)was elevated in 11 cases.Anti-Jo-1 antibody was positive in 4 cases.Bilateral infiltrates including ground-glass attenuation,interlobular septal thickening and reticular opacities were found by chest high resolution computed tomography.The impairment of lung function was manifested as the decreased diffusion capacity for carbon monoxide of the lung(DLCO)and restricted ventilatory function abnormalities.Fifteen cases showed hypoxemia or respiratory failure by arterial blood gas analysis.All patients were treated with glucocorticoid and/or immunosuppressive agents.Totally,14 patients had improved,while 2 patients with no improvement and 4 patients died with a mortality of 20％.Conclusions DM complicated with ILD which is easily missed has a high morbidity and mortality.The understanding of DM complicated with ILD should be enhanced,thus early diagnosis and intervention could be performed for better prognosis.PubMed中文核心期刊要目总览(PKU)中国科技核心期刊(ISTIC)中国科学引文数据库(CSCD)03214-2165