Objectives: Primary Sjögren's syndrome (pSS) is involved in pulmonary tissue. Pulmonary arterial hypertension (PAH) is one of the pulmonary complications caused by pSS. This study aims to investigate the clinical characteristics and risk factors for pSS complicated with PAH.
Methods: We retrospectively analyzed 165 patients in the Second Xiangya Hospital of Central South University. They were divided into a pSS-PAH group (n=86) and a pSS group (n=79) according to pulmonary artery pressure detected by color doppler echocardiography. The clinical characteristics, laboratory test indexes, and risk factors were compared between the 2 groups.
Results: Among 165 patients with pSS, 86 patients (52.12%) had PAH. Females were 79 (91.90%) patients in the pSS-PAH group, more than males. The patients in the pSS-PAH group were older than those in the pSS group (all P<0.05). The incidence of keratoconjunctivitis, alopecia, Raynaud's phenomenon, cough, chest tightness, shortness of breath, and dry skin was higher (all P<0.05), and the incidence of pulmonary infection, pulmonary cystic degeneration, respiratory failure, osteoporosis, arteriosclerosis, and hypertension were higher in the pSS-PAH group than those in the pSS group (all P<0.05). The laboratory indicators of pulmonary artery diameter, right atrium diameter, right ventricular contractile diameter and pulmonary artery systolic pressure were higher in the pSS-PAH group than those in the pSS group (all P<0.05), and the positive rates of anti-nuclear antibody, anti-SSA antibody, and anti-Ro-52 antibody were higher (all P<0.05). The incidence of restrictive ventilatory dysfunction and decreased lung diffusion volume in the pSS-PAH group was higher than that in the pSS group (both P<0.05). Advanced age (OR=1.094, 95% CI 1.053 to 1.137, P<0.001), concomitant keratoconjunctivitis (OR=2.075, 95% CI 1.054 to 4.088, P=0.035), hair loss (OR=2.655, 95% CI 1.368 to 5.152, P=0.004), dry skin (OR=2.696, 95% CI 1.364 to 5.332, P=0.004), high pulmonary artery systolic pressure (OR=1.185, 95% CI 1.125 to 1.248, P<0.001), respiratory failure (OR=2.279, 95% CI 1.137 to 4.570, P=0.020), osteoporosis (OR=2.087, 95% CI 1.025 to 4.248, P=0.043), atherosclerosis (OR=2.251, 95% CI 1.146 to 4.423, P=0.018), hypertension (OR=2.370, 95% CI 1.190 to 4.718, P=0.014), the increased antinuclear antibody (OR=2.155, 95% CI 1.094 to 4.245, P=0.026), the increased anti-SSA antibody (OR=2.565, 95% CI 1.292 to 5.091, P=0.007), the increased anti-RO-52 antibody (OR=2.623, 95% CI 1.278 to 5.383, P=0.009), and the decreased lung dispersion (OR=2.602, 95% CI 1.386 to 4.884, P=0.003), were all risk factors for PAH in pSS patients.
Conclusions: The incidence of pSS-PAH is high in this study. The advanced age, elevated pulmonary artery systolic pressure, concomitant keratoconjunctivitis, alopecia, dry skin, respiratory failure, osteoporosis, arteriosclerosis, and hypertension, increased anti-nuclear antibody, anti-SSA antibody, and anti-Ro-52 antibody, and decreased pulmonary dispersion suggest that the risk of PAH is significantly increased in patients with pSS.
目的: 原发性干燥综合征(primary Sjögren’s syndrome，pSS)可累及肺部组织，肺动脉高压(pulmonary arterial hypertension，PAH)是pSS引起的肺部并发症之一。本研究旨在探讨pSS合并PAH的临床特征和危险因素。方法: 回顾性分析中南大学湘雅二医院165例pSS患者的临床资料，根据患者心脏彩色多普勒超声检查的肺动脉压力分为pSS-PAH组(n=86)和pSS组(n=79)，比较2组间的临床特征和实验室检查指标，分析其危险因素。结果: 165例pSS患者中合并PAH的发生率为52.12%，pSS-PAH组中79例为女性(91.90%)，多于男性。pSS-PAH组的年龄大于pSS组(P<0.05)，角结膜炎、脱发、雷诺现象、咳嗽、胸闷、气促及皮肤干燥的发生率均高于pSS组(均P<0.05)，肺部感染、肺囊性变、呼吸衰竭、骨质疏松症、动脉硬化及高血压的发生率均高于pSS组(均P<0.05)。实验室指标中pSS-PAH组肺动脉内径、右心房内径、右心室收缩内径、肺动脉收缩压均高于pSS组(均P<0.05)，抗核抗体、抗SSA抗体、抗Ro-52抗体的阳性率均高于pSS组(均P<0.05)，限制性通气功能障碍、肺弥散量下降的发生率均高于pSS组(均P<0.05)。高龄(OR=1.094，95% CI 1.053~1.137，P<0.001)，合并角结膜炎(OR=2.075，95% CI 1.054~4.088，P=0.035)、脱发(OR=2.655，95% CI 1.368~5.152，P=0.004)、皮肤干燥(OR=2.696，95% CI 1.364~5.332，P=0.004)、肺动脉收缩压(OR=1.185，95% CI 1.125~1.248，P<0.001)、呼吸衰竭(OR=2.279，95% CI 1.137~4.570，P=0.020)、骨质疏松症(OR=2.087，95% CI 1.025~4.248，P=0.043)、动脉硬化(OR=2.251，95% CI 1.146~4.423，P=0.018)、高血压(OR=2.370，95% CI 1.190~4.718，P=0.014)，抗核抗体升高(OR=2.155，95% CI 1.094~4.245，P=0.026)、抗SSA抗体升高(OR=2.565，95% CI 1.292~5.091，P=0.007)、抗Ro-52抗体升高(OR=2.623，95% CI 1.278~5.383，P=0.009)、肺弥散量下降(OR=2.602，95% CI：1.386~4.884，P=0.003)是pSS患者发生PAH的危险因素。结论: 本研究中pSS-PAH的发生率较高。高龄，肺动脉收缩压升高，合并角结膜炎、脱发、皮肤干燥、呼吸衰竭、骨质疏松症、动脉硬化、高血压，抗核抗体、抗SSA抗体、抗Ro-52抗体升高及肺弥散量下降均提示pSS患者发生PAH的风险明显增加。.
Keywords: clinical features; primary Sjögren’s syndrome; pulmonary arterial hypertension; risk factors.