Rheumatology Division, Medicine Department, Escola Paulista de Medicina, Universidade Federal de São Paulo (UNIFESP), São Paulo, SP, Brazil.
Division of Pneumology, Medicine Department, Escola Paulista de Medicina, Universidade Federal de São Paulo (UNIFESP), São Paulo, SP, Brazil.
Adv Rheumatol. 2019 Jan 6;59(1):1. doi: 10.1186/s42358-018-0045-5.
Pulmonary arterial hypertension (PAH) is a rare and severe complication of systemic lupus erythematosus (SLE). This study aimed to evaluate clinical and laboratory risk factors associated with PAH in SLE patients.
This was a retrospective case-control study in which patients with SLE with PAH (SLE-PAH) confirmed by right heart catheterization (RHC) were compared with SLE patients without PAH. Clinical and demographic variables related to SLE and PAH and nailfold capillaroscopy were evaluated by reviewing the medical records of the patients.
Twenty-one patients with SLE-PAH and 44 patients with SLE without PAH matched for sex and disease duration were included. The scleroderma (SD) pattern on nailfold capillaroscopy was more frequently found in patients with SLE-PAH than in those without PAH (56.3% versus 15.9%, respectively, p = 0.002). By univariate analysis, Raynaud's phenomenon, history of abortion, and SD pattern on capillaroscopy were associated with PAH. Arthritis was a protective factor for PAH development. Multivariate analysis showed that the SD pattern on capillaroscopy was the only variable associated with a significantly higher risk of PAH, with an odds ratio of 6.393 (95% confidence interval, 1.530-26.716; p = 0.011).
In this study, SD pattern was associated with a 6.3-fold increased risk for PAH development in SLE patients, suggesting that nailfold capillaroscopy might be useful as a screening method to identify SLE patients with a high risk of developing this severe complication.
肺动脉高压（PAH）是系统性红斑狼疮（SLE）的一种罕见且严重的并发症。本研究旨在评估与 SLE 患者 PAH 相关的临床和实验室危险因素。
这是一项回顾性病例对照研究，通过右心导管检查（RHC）确诊的 SLE 合并 PAH（SLE-PAH）患者与无 PAH 的 SLE 患者进行比较。通过回顾患者的病历，评估与 SLE 和 PAH 相关的临床和人口统计学变量以及甲襞毛细血管镜。
共纳入 21 例 SLE-PAH 患者和 44 例 SLE 无 PAH 患者，两组在性别和疾病持续时间上相匹配。与无 PAH 的患者相比，SLE-PAH 患者更常出现甲襞毛细血管镜下的硬皮病（SD）模式（分别为 56.3%和 15.9%，p=0.002）。单因素分析显示，雷诺现象、流产史和毛细血管镜下的 SD 模式与 PAH 相关。关节炎是 PAH 发病的保护因素。多因素分析显示，毛细血管镜下的 SD 模式是唯一与 PAH 发生风险显著增加相关的变量，优势比为 6.393（95%置信区间，1.530-26.716；p=0.011）。
在这项研究中，SD 模式与 SLE 患者 PAH 发展的风险增加 6.3 倍相关，提示甲襞毛细血管镜可能是一种有用的筛查方法，可用于识别发生这种严重并发症风险较高的 SLE 患者。
