混合性结缔组织病相关肺动脉高压临床特点分析

Analysis of clinical features of mixed connective tissue disease associated with pulmonary arterial hypertension

目的探究混合性结缔组织病相关肺动脉高压(MCTD-PAH)患者的临床特点及发病危险因素。方法回顾性纳入12例住院治疗的MCTD-PAH患者(MCTD-PAH组),根据性别、年龄按1︰3随机抽取同期住院的36例混合性结缔组织病无肺动脉高压(MCTD-non-PAH)患者作为对照组,比较2组患者的临床表现和辅助检查,随诊2组患者生存状态。结果MCTD-PAH组较对照组出现活动后气短、肌炎及心包积液比例更高,血沉及免疫球蛋白G(IgG)水平更高。多因素Logistic回归分析显示,活动后气短及较高水平的IgG是预测MCTD发生PAH的危险因素。MCTD-PAH死亡3例(16.7%),对照组无患者死亡。结论PAH是MCTD严重的并发症之一,MCTD患者出现活动后气短及较高水平的IgG时需警惕合并PAH。

Objective To investigate the clinical characteristics and risk factors of mixed connective tissue disease associated with pulmonary arterial hypertension(MCTD-PAH).Methods Twelve MCTD-PAH patients diagnosed by right heart catheterization(RHC)at Tianjin Medical University General Hospital were retrospectively included,and 36 MCTD patients without pulmonary arterial hypertension(MCTD-non-PAH)were randomly selected from the same period of hospitalization based on gender and age.The clinical features and auxiliary examination of the two groups were compared,and the survival status of the two groups was compared.Results The proportion of dyspnea after activity,myositis and pericardial effusion were higher in the MCTD-PAH group than those of the control group.Serum sedimentation rate and immunoglobulin G(IgG)levels were higher in the MCTD-PAH group.Multivariate Logistic regression analysis showed that dyspnea after activity and high level of IgG were risk factors for predicting the occurrence of PAH in MCTD.Three patients(16.7%)died in the MCTD-PAH group,and no patients died in the control group.Conclusion Pulmonary arterial hypertension is one of the serious complications of MCTD.MCTD patients have shortness of breath after activity and high level of IgG should be wary of concomitant PAH.