130例系统性硬化病患者肺动脉高压的检测及其高危因素分析  

Detection of pulmonary hypertension in 130 systemic sclerosis patients and analysis of high risk factors

作　　者：崔祥祥 杨骥 屠文震[3] 李明 CUI Xiang-xiang;YANG Ji;TU Wen-zhen;LI Ming(Department of Dermatology,Children’s Hospital of Shanghai,Shanghai 200062,China)

机构地区：[1]上海市儿童医院皮肤科,上海200062 [2]复旦大学附属中山医院皮肤科,上海200032 [3]上海市中西医结合医院硬皮病专科,上海200082

出　　处：《临床皮肤科杂志》2022年第4期204-207,共4页Journal of Clinical Dermatology

基　　金：国家自然科学基金(81573043)资助项目。

摘　　要：目的:分析系统性硬化病(SSc)并发肺动脉高压(pulmonary arterial hypertension,PAH)(SSc-PAH)患者的高危因素。方法:收集2012—2016年复旦大学附属中山医院皮肤科及上海市中西医结合医院硬皮病专科确诊的130例SSc患者,按其有无PAH分为SSc-PAH组和SSc无PAH(SSc-no PAH)组,并对这两组患者的临床特征进行分析。将弥漫型系统性硬化病并发肺动脉高压(dcSSc-PAH)组和弥漫型系统性硬化病无肺动脉高压(dcSSc-no PAH)组按病程各分为两组(A组<3年,B组≥3年)。局限型系统性硬化病并发肺动脉高压(lcSSc-PAH)组和局限型系统性硬化病无肺动脉高压(lcSSc-no PAH)组按病程各分为2组(C组<5年,D组≥5年),分别统计PAH在各组中的发生率。结果:130例SSc患者中,SSc-PAH 27例(20.8%)。27例SSc-PAH患者皮肤损害发生率依次为:雷诺现象26例(96.3%);手指硬化24例(88.9%);手指肿胀19例(70.4%);皮肤异色症19例(70.4%);毛细血管扩张16例(59.3%);面具貌13例(48.1%);指尖凹陷6例(22.2%);指腹萎缩5例(18.5%);指尖溃疡2例(7.4%)。确诊SSc-PAH时发生雷诺现象的时间为(5.41±7.68)年。DcSSc-PAH在A组和B组的发生率分别为46.2%和53.8%,lcSSc-PAH在C组和D组的发生率分别为58.3%和41.7%。DcSSc-PAH在A组和B组的发生率和lcSSc-PAH在C组和D组的发生率差异均无统计学意义。27例SSc-PAH患者中,抗DNA拓扑异构酶Ⅰ(SCL-70)抗体阳性6例(22.2%);抗着丝点抗体(ACA)阳性12例(44.4%);抗干燥综合征A(SSA)抗体阳性9例(33.3%);抗干燥综合征B(SSB)抗体阳性3例(11.1%)。27例SSc-PAH患者中器官累及发生率依次为心脏累及14例(51.9%);肺间质纤维化12例(44.4%);关节累及9例(33.3%);吞咽困难8例(29.6%);肾脏7例(25.9%)。SSc-PAH组ACA阳性率高于SSc-no PAH组(χ^(2)=5.317,P<0.05)。SSc-PAH组心脏累及与肾脏累及发生率高于SSc-no PAH组(χ^(2)值分别为13.623、6.911,P均<0.01)。结论:SSc患者雷诺现象发生(5.41±7.68)年后易出现PAH。PAH在SSc早期就会出现,Objective:To explore and analyze the high risk factors in patients with systemic sclerosis complicated with pulmonary arterial hypertension(PAH).Methods:Data of 130 SSc patients diagnosed in the Dermatology Department of Zhongshan Hospital and Department of Scleroderma of Shanghai TCM-Integrated Hospital from 2012 to 2016 were collected and divided into SSc-PAH group and SSc-no PAH group according to whether they had PAH.The clinical characteristics of these two groups were analyzed.DcSSc-PAH(diffuse systemic sclerosis with pulmonary arterial hypertension)group and dcSSc-no PAH(diffuse systemic sclerosis without pulmonary arterial hypertension)group were further divided into two groups according to the course of disease(group A less than 3 years,group B 3 years or more),respectively.The lcSSc-PAH(localized systemic sclerosis with pulmonary arterial hypertension)group and lcSSc-no PAH(localized systemic sclerosis without pulmonary arterial hypertension)group were divided into two groups according to the course of disease(group C less than 5 years and group D 5 years or more),respectively.The incidence of PAH in each group was calculated.Results:Among the 130 SSc patients,27(20.8%)had PAH.Of the 27 SSc-PAH patients,26(96.3%)developed Raynaud’s phenomenon,24(88.9%)had sclerosis of finger dorsum,19(70.4%)suffered swelling of finger,19(70.4%)had poikiloderma,16(59.3%)had telangiectasia,13(48.1%)had mask face,6(22.2%)had depressed fingertip,5(18.5%)had atrophy of the finger pulp,and 2(7.4%)had fingertip ulcer.The duration of Raynaud’s phenomenon till the time of SSc-PAH diagnosis was 5.41±7.68 years.The incidence of dcSSc-PAH in group A and group B was 46.2%and 53.8%,respectively,while the incidence of lcSSc-PAH in group C and group D was 58.3%and 41.7%,respectively.There was no significant difference in the incidence of dcSSc-PAH in group A and group B,nor in the incidence of lcSSc-PAH in group C and group D.Of the 27 SSc-PAH patients,anti-DNA topoisomeraseⅠ(SCL-70)anti body and anticentromere antibody(ACA)were

关 键 词：硬皮病 系统性 高血压 肺性 皮肤表现 自身抗体 器官累及 

分 类 号：R593.25[医药卫生—内科学]