翻译者：谢荣华，西京医院临床免疫科

目  的:

目的探讨原发性干燥综合征（pSS）患者右心导管插入术（RHC）证实的肺动脉高压（PAH）的基线特征，探讨pSS中PAH的危险因素。

方  法:

该病例对照研究以2007年至2015年住院的pSS-PAH患者为研究对象，无PAH的pSS患者（比例为4:1）作为对照。所有患者均符合2002年欧美共识组pSS-PAH分类标准，该标准是基于RHC欧洲心脏病学会/欧洲呼吸学会指南确定。用单变量二元logistic回归分析相关变量，以确定PAH的可能危险因素。

结  果:

总共29例经RHC证实的 pSS-PAH（包括平均发病年龄，38.4±8.3年；平均pSS持续时间54.6个月）患者纳入研究。12例pSS患者初始表现为肺动脉高压是(41.4%)，气短是最常见的症状(29/29，100%)。平均肺动脉压力为51.8±10.0mmHg，心脏指数是2.3±0.8 L/min/m2，肺血管阻力是13.0±6.0Wood单位。治疗包括免疫抑制剂（93.1%）和PAH靶向治疗(86.2%)。研究发现了四个独立的pSS-PAH风险因素：包括雷诺氏现象（OR= 9.660，P = 0.000），类风湿因子≥200 U /ml （OR= 6.691，P = 0.001），肝脏损伤（OR= 3.284，P = 0.008）和心包积液（OR= 3.279，P = 0.016）。

结  论:

PAH可能是pSS的首发表现，pSS患者出现雷诺症、高滴度类风湿因子、肝损伤或心包积液时应早期筛查肺动脉高压。

参考文献：

Int J Rheum Dis. 2018 May;21(5):1068-1075. Epub2018 Apr 6.

Characteristics and risk factorsof pulmonary arterial hypertension in patients with primary Sjögren's syndrome.

Yan S1,2, Li M2, Wang H3, Yang X2, Zhao J2, Wang Q2, Liu Y3, Lai J3, Tian Z3, Song H1, Zhao Y2, Zeng X2.

Abstract

AIM:

Todescribe baseline characteristics of patients with primary Sjögren's syndrome(pSS) with right heart catheterization (RHC)-confirmed pulmonary arterialhypertension (PAH) and explore risk factors for PAH in pSS.

METHODS:

Thiscase-control study included consecutive patients hospitalized with pSS-PAH from2007 to 2015, and pSS patients without PAH (in a 4 : 1 ratio) as controls. Allpatients fulfilled the 2002 American-European Consensus Group classificationcriteria for pSS-PAH was defined according to RHC-based European Society ofCardiology/European Respiratory Society guidelines. Associated variables wereanalyzed by univariate binary logistic regression to identify possible riskfactors for PAH.

RESULTS:

Twenty-ninepatients with RHC-confirmed pSS-PAH were included (mean age at onset, 38.4 ±8.3 years; mean pSS duration, 54.6 months). PAH was the initial manifestationof pSS in 12 patients (41.4%), and shortness of breath was the most commonsymptom (29/29, 100%). Mean pulmonary arterial pressure was 51.8 ± 10.0 mmHg,mean cardiac index was 2.3 ± 0.8 L/min/m2 , and mean pulmonary vascularresistance was 13.0 ± 6.0 Wood units in this group. Treatments includedimmunosuppressive agents (93.1%) and PAH-targeted therapies (86.2%). Weidentified four independent risk factors for PAH in pSS: Raynaud's phenomenon(odds ratio [OR] = 9.660, P = 0.000), rheumatoid factor ≥ 200 U/mL (OR = 6.691,P = 0.001), hepatic injury (OR = 3.284, P = 0.008) and pericardial effusion (OR= 3.279, P = 0.016).

CONCLUSIONS:

PAH can be the firstmanifestation of pSS. The pSS patients with Raynaud's phenomenon, high-titerrheumatoid factor, hepatic injury or pericardial effusion should be screenedfor PAH.