皮肌炎患者病死原因分析

Analysis of causes of death in patients with dermatomyositis

目的：探讨导致皮肌炎（dermatomyositis,DM）患者病死的危险因素。方法：对156例DM患者的临床资料进行回顾性分析,其中病死44例。结果：病死率为22.2%,男性39.6%高于女性13.5%（P〈0.01）,病死者的发病年龄高峰是56～65岁,急性发病和亚急性发病的病死率分别为33.3%、29.5%均高于慢性发病者的12.3%（P〈0.05）。44例病死者中,病程〈1年者29例,病程〈3年37例（84.1%）的构成比大于病程≥3年者。18例伴恶性肿瘤,18例有肺间质病变（ILD）中5例继发肺部感染,6例死于肺部感染,2例死因不明。高危组DM患者血清γ-谷氨酰转移酶（GGT）、乳酸脱氢酶（LDH）、红细胞沉降率（ESR）和高丙种球蛋白升高率均高于良性组（P〈0.05～P〈0.01）。高危组关节痛、发热、心电图异常与良性组差异有统计学意义（P〈0.01）。血清肌酸激酶、抗核抗体、抗dsDNA抗体、抗Jo-1抗体水平和DM的皮疹类型等与良性组差异均无统计学意义（P〉0.05）。结论：DM患者的病死多发生在病程的3年内,尤其是第1年内;导致DM患者病死的危险因素主要是恶性肿瘤、ILD、肺部感染、急性和亚急性起病、年龄、男性、关节痛、发热、心电图异常;血清GGT、LDH、ESR和高丙种球蛋白对预测DM的预后有重要价值。

Objective：To investigate the risk factors leading to death for patients with dermatomyositis（DM）.Methods：The clinical data of 156 patients with DM including 44 patients who had died were analyzed retrospectively.Results：The mortality rate was 22.2%,with the male accounting for 39.6% and the female 13.5%（P〈0.01）.The peak age of onset ranged from 56 to 65.The mortality rate in cases with acute onset and subacute onset was 33.3% and 29.5%,respectively,which was higher than that with chronic onset（12.3%）（P〈0.05）.Among the 44 cases who had died,29 cases had a disease course of less than 1 year and 37 cases less than 3 years,the mortality of which was higher than that over 3 years;18 of the cases were complicated with malignant tumors and 18 interstitial lung disease（ILD）（including 5 secondary infections） ;6 cases died of pulmonary infection and 2 unknown causes.The serum level of lactate dehydrogenase（LDH）,γ-glutamyltransferase（GGT）,erythrocyte sedimentation rate（ESR） and gamma globulin were higher in the patients with poor outcome than that in those with fine outcome（P〈0.05-P〈0.01）.There were significant difference in arthralgia,fever and electrocardiographic abnormality between patients with poor outcome and fine outcome（P〈0.01）.The differences in serum levels of CK,positive anti-ANA,positive anti-dsDNA,positive anti-Jo-1 antibody and the skin rashes were not significant between the two patient groups（P〉0.05）.Conclusions：These data indicate that the mortality of dermatomyositis occur mostly in the first 3 years of onset,especially the first year.The risk factors leading to death for DM patients are malignancy,ILD,pulmonary infection,acute and subacute onsets,old age,maleness,arthralgia,fever and electrocardiographic abnormality.The serum levels of GGT,LDH,ESR and gamma globulin have prognostic value for the DM patients.