皮肌炎/多发性肌炎合并肺间质病变的临床特征及预后相关因素

Clinical features of dermatomyositis/polymyositis complicated with interstitial lung disease

目的探讨皮肌炎/多发性肌炎(DM/PM)合并肺间质病变(ILD)的临床特征和预后。方法回顾住院诊治及门诊长期随访的141例DM/PM患者的临床资料及实验室指标,对PM/DM合并ILD的生存时间、预测因素和预后不良因素进行分析。结果141例DM/PM患者中合并ILD 42例(29.8%),其1、5、10年生存率分别为76%、66%和57%,中位生存时间为127.3个月。多因素Cox回归分析得出:合并ILD、年龄>50岁、声音嘶哑、低白蛋白血症、低氧血症是DM/PM预后不良的独立危险因素(均P<0.05)。logistic回归显示,低白蛋白血症、技工手和抗Jo-1抗体与ILD呈正相关(均P<0.05),成为ILD相关预测因子。急性/亚急性ILD进展快,病死率高(75%)。丙种球蛋白治疗与病死率呈负相关(P<0.05)。结论ILD是DM/PM的常见并发症,也是影响预后的危险因素。技工手、抗Jo-1抗体和低白蛋白血症是DM/PM合并ILD的预测因子,低氧血症是影响ILD预后的不良因素,早期使用丙种球蛋白冲击治疗有助改善预后。

Objective To review the clinical characteristics and prognosis of dermatomyositis （DM） and polymyositis （PM） complicated with interstitial lung disease （ILD）. Methods The clinical and laboratory data were col ected from 141 patients with DM or PM admitted to Ningbo First Hospital from January 1997 to August 2012.The survival, predicting and prognostic factors of PM/DM patients complicated with ILD were analyzed retrospectively. Results Forty- two out of 141 DM/PM patients were com-plicated with ILD（29.8%）.The 1, 5, 10- years survival rates were 76%, 66%and 57%, respectively, with a median survival time of 127.3 months. The presence of clinical ILD and the hyoxemia were the risk factors for DM/PM （OR=2.536, 95%CI 1.205- 5.335, P=0.014 and OR=16.572, 95%CI 3.238- 84.817, P=0.001, respectively）. Logistic regression showed that anti- Jo- 1 antibody, me-chanic＇s hand and hypoproteinemia were positively correlated with ILD in DM/PM.DM/PM complicated by acute or subacute in-terstitial pneumonia progressed rapidly with a high mortality （75%）. Treatment with intravenous immuuoglobulin was negatively correlated with mortality in DM/PM- ILD patients （r=- 0.331, P=0.032）. Conclusion ILD is a common complication of DM/PM and is also a prognostic factor. The DM/PM patients with anti- Jo- 1 antibody, mechanic＇s hand and hypoproteinemia tend to compli-cate with ILD. Hyoxemia is a risk factor of poor prognostic in patients with DM/PM- ILD. Early immuuoglobulin treatment might improve the prognosis of DM/PM- ILD.