系统性硬化患者发生重度肺动脉高压的危险因素分析

目的分析预测系统性硬化患者发生重度肺动脉高压（SPAH）的危险因素。方法收集146例系统性硬化患者的临床资料，包括起病年龄、性别、病程、雷诺现象（RP）、皮肤受累程度、胃食管反流、心脏损害、心功能不全、肾损害、肺动脉压、间质性肺炎、弥散功能、脑梗死、血清抗Scl-70抗体、抗RNP抗体和抗着丝点抗体情况。使用非参数检验方法比较SPAH组和无SPAH组之间临床指标的差异．并使用Cox回归方法分析患者发生SPAH的危险因素。结果SPAH组与无SPAH组患者相比，RP、男性患者、抗Scl-70、抗着丝点抗体、间质性肺炎、弥散功能下降、胃食管反流、冠心病、外周动脉硬化发生率均无显著性差异（P〉0．05）；SPAH组患者中起病年龄〉60岁者、弥漫型皮肤受累（DSSc）者、抗RNP抗体阳性者、心脏损害者、肾脏损害者、脑梗死者均多于无重度肺动脉高压者（分别为44．4％vs．12．5％、55．6％vs．21．9％、44．4％vs．15．6％、44．4％vs．14．1％、33．3％vs．9．4％、11．1％vs．1．6％）。Cox回归分析表明。发病年龄〉60岁（OR=7．091，95％可信区间2．633～19．095，P=0．000）、男性（OR=3．833,95％可信区间1．200～12．246．P=0．023）、RNP抗体阳性（OR=3．031，95％可信区间1．163～7．904，P=0．023）、DSSc（OR=2．635,95％可信区间1．031—6．737，P=0．043）、心脏受累（OR=3．284，95％可信区间1．261～8．553，P=0．015）、肾脏受累（OR=3．089，95％可信区间1．096-8．708，P=0．033）是系统性硬化患者发生SPAH的危险因素。结论对于发病年龄〉60岁、男性、DSSe、RNP抗体阳性、心脏受累、肾脏受累患者应早期监测其肺动脉压，以期早期检出肺动脉高压并积极治疗。

Risk of developing severe pulmonary artery hypertension in systemic sclerosis

Objective To determine the risk factors that might predict the occurrence of severe pulmonary artery hypertension in systemic sclerosis patients.Methods Clinical data of patients with definite systemic sclerosis were collected,including disease onset age,sex,disease course,Raynaud's phenomenon,skin involvement,gastroesophageal reflux,cardiac lesions,kidney lesions,cerebral infarct,interstitial pneumonia,alveolar diffusion capacity,serum antibodies to Scl-70,RNP and anti-centromere antibodies.Pulmonary artery pressure was measured by doppler echocardiography.Mann-Whitney U test were employed to compare the clinical profiles between patients with severe pulmonary artery hypertension and those without.Cox hazard ratio model was used to assess the risk factors for developing severe pulmonary artery hypertension in systemic sclerosis patients.Results No difference in sex,Raynaud's phenomenon,anti-Scl 70 antibody,anti-centromere antibody,interstitial pneumonia,diffusion capacity,gastroesophageal reflux,coronary artery disease,and peripheral artery atherosclerosis was found between systemic sclerosis patients with pulmonary artery hypertension and those without(P>0.05).As compared to those without pulmonary artery hypertension,patients with severe pulmonary artery hypertension had more disease onset later than 60 years old(P=0.001),more diffuse skin involvement(P=0.003),more frequent positive anti-RNP antibody(P=0.005),more cardiac lesions(P=0.002),more cerebral infarct(P=0.035),and more kidney lesions(P=0.004).Cox regression anaylysis showed that,onset later than 60 years old (OR=7.091,95%CI 2.633～19.095,P=0.000),male sex(OR=3.833,95%CI 1.200～12.246,P=0.023),RNP antibody positivity(OR=3.031,95%CI 1.163～7.904,P=0.023),diffuse skin involvement(OR=2.635,95%CI 1.031～6.737,P=0.043),cardiac lesions(OR=3.284,95%CI 1.261～8.553,P=0.015)and kidney lesions(OR=3.089,95%CI 1.096～8.708,P=0.033)were risk factors for developing severe pulmonary artery hypertension in systemic sclerosis patients.Conclusions Close monitoring of pulmonary artery pressure by doppler echocardiography should be done in systemic sclerosis patients with onset later than 60 years old,male,positive RNP antibody,cadiac involvement and kidney lesions.