系统性红斑狼疮相关肺动脉高压

Systemic lupus erythematosus associated pulmonary arterial hypertension

系统性红斑狼疮(SLE)是一种以多器官系统受累为主要特征的自身免疫性疾病,肺动脉高压(PAH)是其常见和严重并发症。中国导致PAH最常见的结缔组织病是SLE,自身免疫反应及炎症是其发生和进展的主要因素。临床表现包括SLE本身和PAH两个方面。SLE相关PAH患者的预后较其他结缔组织病相关PAH患者更好,因为针对原发病SLE的免疫抑制治疗有效。针对PAH的靶向药物对于SLE相关PAH的治疗有效,尤其用于免疫制剂治疗无效的患者。早期发现和恰当治疗是改善患者预后的关键。

Systemic lupus erythematosus （SLE） is an autoimmune disease characterized by multiple organ involvement. Pulmonary arterial hypertension （PAH） is a common and severe complication ofSLE. SLE is the most common connective tissue disease that leads to PAH in Chinese. Autoimmune inflammation triggers the onset and development of SLE associated PAH. These patients manifest the features of SLE as well as PAH. The prognosis of patients with SLE associated PAH is usually better than those PAH secondary to other connective tissue disease because most of the patients with SLE associated PAH respond to immnnosuppressive therapy： Those targeted medications work for patients with SLE associated PAH, especially those who failed to immunosuppressive therapy： Early diagnosis and appropriate intervention are key to improve the prognosis.