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目的探讨皮肌炎(DM)并发间质性肺病(ILD)的临床特点和意义。方法收集北京协和医院1970-1997年门诊和住院长期随诊的216例DM患者,符合Bohan和Peter1975年诊断标准。随诊2~27年,平均(5.8±4.7)年。DM合并ILD32例,DM无ILD184例。比较两组临床资料和实验室各指标,以这些参数作统计学分析。结果DM患者中,ILD组初发症状中发热(28.2%)显著多于无ILD组(6.5%),P<0.01。病程中ILD组的发热、关节炎、皮肤溃疡和类风湿因子阳性明显增高,分别为71.9%、62.5%、31.3%和24.1%,无ILD组分别为34.2%、8.7%、8.1%,两组比较,P均<0.01或<0.05。而两组患者眼睑紫红斑、Gottron征、吞咽困难、肌肉痛、肌无力、抗核抗体阳性和肌电图肌源性改变发生率差异均无显著性(P均>0.05)。ILD组32例中死亡13例(40.6%),病情改善15例(46.9%),缓解而停用皮质类固醇4例(12.5%)。184例DM无ILD者死亡30例(16.3%),两组死亡率差异有显著性(P<0.01)。结论DM患者有ILD者发热、关节炎、皮肤溃疡和类风湿因子阳性明显增多。这组患者治疗困难,死亡率高。
Objective To investigate the clinical features and significance of dermatomyositis (DM) complicated by interstitial lung disease (ILD). Methods A total of 216 DM patients were selected from Peking Union Medical College Hospital from 1970 to 1997 in long-term follow-up and hospitalization, which met the diagnostic criteria of Bohan and Peter in 1975. Followed up for 2 to 27 years, with an average of (5.8 ± 4.7) years. DM combined ILD32 cases, DM no ILD184 cases. The two groups of clinical data and laboratory indicators were compared, with these parameters for statistical analysis. Results In patients with DM, the incidence of initial fever in the ILD group (28.2%) was significantly higher than that in the non-ILD group (6.5%) (P <0.01). ILD group had significantly higher fever, arthritis, skin ulcer and rheumatoid factor in the course of the disease, which were 71.9%, 62.5%, 31.3% and 24.1% respectively in the ILD group, 34.2%, 8.7% and 8.1% in the non-ILD group P <0.01 or <0.05, respectively. There was no significant difference in the incidence of edema, Gottron sign, dysphagia, muscle pain, muscle weakness, antinuclear antibody positive and EMG myogenic changes between the two groups (all P> 0.05). Among 32 patients in ILD group, 13 patients died (40.6%), 15 patients (46.9%) improved and 4 patients (12.5%) were relieved and corticosteroids were discontinued. 184 cases of DM without ILD died in 30 cases (16.3%), the difference between the two groups was significant (P <0.01). Conclusion DM patients with ILD fever, arthritis, skin ulcers and rheumatoid factor was significantly increased. This group of patients with difficult treatment, high mortality.