Department of Epidemiology and Biostatistics, School of Public Health, Anhui Medical University, 81 Meishan Road, Hefei, 230032, Anhui, People's Republic of China.
Anhui Province Key Laboratory of Major Autoimmune Diseases, 81 Meishan Road, Hefei, Anhui, 230032, China.
Clin Rheumatol. 2018 Jun;37(6):1547-1553. doi: 10.1007/s10067-018-4056-8. Epub 2018 Mar 8.
Pulmonary arterial hypertension (PAH) is an increasingly recognized complication of systemic lupus erythematosus (SLE). This study aims to estimate the point prevalence of PAH and identify risk factors for PAH in a large cohort of hospitalized SLE patients. We have collected the medical records of patients hospitalized with SLE at the First Affiliated Hospital of Anhui Medical University and Anhui Provincial Hospital. Resting transthoracic echocardiography (TTE) was used to estimate pulmonary artery pressure (PAP) and PAH was defined as systolic PAP (PASP) > 30 mmHg. Patients with other connective tissue diseases, aPL syndrome, left heart disease, valvular heart disease, congenital heart disease, HIV, and portal hypertension were excluded because of diseases affecting the PAP. We assessed potential risk factors for PAH such as thrombogenic factors, SLE clinical manifestations, laboratory abnormalities and disease activity. Ninety-five were diagnosed with PAH of 1639 patients with SLE. The presence of high fibrinogen, serositis, and thrombocytopenia were significantly higher in patients with PAH than in those without PAH (all P < 0.05). Multivariate logistic regression found the associations between high fibrinogen (OR = 1.629), serositis (OR = 2.866), and thrombocytopenia (OR = 1.825) with PAH. The point prevalence of PAH was 5.8% in our cohort of patients with SLE. The significant association of high fibrinogen, serositis, and thrombocytopenia with PAH suggested that hypercoagulable state, organ damage, and hematological abnormality may all contribute to the development of PAH in SLE. This is important, as it is treatable.
特发性肺动脉高压(PAH)是系统性红斑狼疮(SLE)的一种日益被认识的并发症。本研究旨在评估住院 SLE 患者中 PAH 的现患率并确定其危险因素。我们收集了安徽医科大学第一附属医院和安徽省立医院住院 SLE 患者的病历。采用静息经胸超声心动图(TTE)来估计肺动脉压(PAP),将收缩期 PAP(PASP)>30mmHg 定义为 PAH。由于 PAP 受其他影响的疾病影响,排除患有其他结缔组织病、抗磷脂综合征、左心疾病、瓣膜性心脏病、先天性心脏病、HIV 和门静脉高压的患者。我们评估了血栓形成因素、SLE 临床表现、实验室异常和疾病活动度等可能导致 PAH 的潜在危险因素。在 1639 例 SLE 患者中,有 95 例诊断为 PAH。PAH 患者的高纤维蛋白原、浆膜炎和血小板减少症的发生率明显高于无 PAH 患者(均 P<0.05)。多变量逻辑回归发现,高纤维蛋白原(OR=1.629)、浆膜炎(OR=2.866)和血小板减少症(OR=1.825)与 PAH 之间存在关联。本研究中 SLE 患者的 PAH 现患率为 5.8%。高纤维蛋白原、浆膜炎和血小板减少症与 PAH 的显著相关性提示,高凝状态、器官损害和血液学异常可能都有助于 SLE 中 PAH 的发生。这一点很重要,因为它是可以治疗的。