结缔组织病合并肺动脉高压及肺间质病变的肺功能分析

A study, on the abnormality of pulmonary function test of connective tissue diseases associated pulmo- nary arterial hypertension and interstitial lung disease

目的比较和分析不同结缔组织病（CTD）相关肺部病变患者组间各项肺功能指标的差异，探讨肺功能检查对于评价CTD肺部病变性质和程度的临床意义。方法前瞻性纳入CTD肺脏受累患者，根据肺部病变性质分为3组：CTD合并肺动脉高压组（CTD—PAH）29例、合并肺问质病变组（CTD—ILD）35例、合并PAH及ILD组（CTD—PAH＋ILD）16例；另纳入无肺部病变的CTD患者34例作为对照组。比较各组间肺总量占预计值百分比（TLC％）、用力肺活量占预计值百分比（FVC％）、第1秒用力呼气量占预计值百分比（FEV1.0％）、FEV1.0/FVC、一氧化碳弥散量占预计值百分比（DLco％）、弥散率（DLco／VA）等主要肺功能指标的差异。结果共114例患者纳入本研究，以女性多见，平均年龄35～39岁，易合并肺部病变的CTD依次为：混合性结缔组织病（MCTD）、系统性硬化症（SSc）、系统性红斑狼疮（SLE）和原发性干燥综合征（pSS）。CTD—PAH组、CTD—ILD组和CTD—PAH＋ILD组分别有10％、29％和46％的患者出现TLC下降；分别有50％、36％和71％的患者出现FVC％下降；分别有54％、47％和71％的患者出现FEV，膨下降；分别有100％、82％和100％的患者出现DLco％下降。多组间样本比较分析发现TLC％、FVC％、FEV1.0％、DLco％在CTD对照组与CTD合并各肺部病变组之间的差异均有统计学意义（P〈0．05），而在CTD—ILD组与CTD—PAH组间差异均无统计学意义。TLC％在CTD—PAH组大于CTD—PAH＋ILD组[（89±15）％与（79,12）％，P〈0．05）]；FVC％在CTD—PAH组或CTD—ILD组均大于CTD—PAH＋ILD组[（81±13）％、（80±16）％与（65±22）％，P〈0．05）]。结论肺功能检查对于筛查CTD合并的各种肺部病变具有临床应用价值，常表现为限制性通气功能障碍和弥散功能障碍，但从单次数值上无法鉴别CTD肺部病变种类（PAH与ILD）。

Objective To determine the clinical significance of pulmonary function test （PFT） in evaluating the features and severity of lung impairments associated with connective tissue diseases （CTD） by comparing the differences of pulmonary function test parameters among groups of CTD associated pulmonary disorders. Methods Cases of CTD associated pulmonary disorders were prospectively enrolled and assigned into 3 groups according to their lung impairments： CTD associated pulmonary arterial hypertension group （CTD-PAH, n=29）, CTD associated interstitial lung disease group （CTD-ILD, n=35）, CTD associated PAH plus ILD group （CTD-PAH＋ILD, n=16 ） and CTD control group （n=34）. Pulmonary function test parameters, including total lung capacity （TLC % predicted）, forced vital capacity （FVC % predicted）, forced expiratory volume in the first second （FEV1.0% predicted）, FE1.0%/FVC and diffusing capacity of the lung for carbon monoxide （DLco, % predicted） were measured and compared among the four groups. Results One hundred and forteen cases were included and predominantly female with average onset age of 35-39 years old. CTDs that were predisposed to lung diseases were mixed connective disease （MCTD）, systemic sclerosis （SSe）, systemic lupus erythematosus （SLE） and primary Sjoren syndrome （pSS）, in order. There were 10, 29 and 46 percent of patients presented with decreased TLC% in CTD-PAH, CTD-ILD and CTD-PAH ＋ILD group respectively, 50, 36 and 71 pereent of patients with decreased FVC% respectively, 54, 47 and 71 percent of patients with decreased FEV1.0% respectively, and 100, 82 and 100 percent with decreased DLco% respectively. ANOVA analysis demonstrated that TLC%, FVC%, FEVL0%, DLco% had significant differences between CTD control group and each of the CTD associated lung disease group （P〈O.05）, although none of them was lack of difference between the PAH and ILD groups. TLC% was significantly higher in CTD-PAH group than CTD-PAH＋ILD group [ （89±15）% vs （79±12）%,P〈0.05 ], while FVC% was significantly lower in CTD- PAH＋ILD group either than CTD-PAH group or than CTD-ILD group [（81±13）%, （80±16）% vs （65±22）%,P〈0.05]. Conclusion Pulmonary function test may be valuable in early screening for CTD associated lung disordes than distinguishing CTD-PAH from ILD, which usually reveal restrictive ventilation dysfunction and/or diffusing capacity dysfunction.