结缔组织病相关肺动脉高压的早期诊断与治疗

肺动脉高压是结缔组织病患者最为严重的并发症之一,影响到最多达15%的结缔组织病患者,预后很差,已有证据显示在结缔组织病患者中早期发现肺动脉高压进而早期开始治疗能够改善患者的预后。除常用的超声心动图外,近年来已有一些生化标记物、肺功能检查及心电图指标等被发现有助于早期筛查肺动脉高压,也有一些联合多种参数的筛查流程问世。治疗方面肺动脉靶向药物包括内皮素受体拮抗剂、前列环素类似物以及磷酸二酯酶-5抑制剂均被证实对于治疗结缔组织病相关肺动脉高压有效。本综述的目的是描述结缔组织病相关肺动脉高压的发病及临床特点、早期诊断及治疗,着重强调最新发表的一系列早期筛查方法以及已经被证实的治疗药物。

Early detection and treatment of connective tissue disease associated pulmonary arterial hypertension

Pulmonary arterial hypertension （PAH） which can affect up to 15% of patients with connective tissue disease （CTD） is a severe complication of CTD and has a dramatic impact on prognosis and survival. Despite increasing recognition of PAH in CTD, the diagnosis is often delayed, which may lead to unfavorable outcomes in these patients. Recent evidence indicates that the earlier treatment is started in the course of disease, the better the prognosis. Therefore, early detection of PAH has become an important consideration in the optimal management of patients with CTD.Within recent years, more and more biomarkers such as N-terminal pro-brain natriuretic peptide （NT-ProBNP）, pulmonary function parameters such as forced vital capacity/diffuse capacity for carbon monoxide （FVC/DLCO） and electrocardiogram have been found to be useful for detecting PAH earlier, and some algorithms such as DETECT algorithm for early detection of PAH in systemic sclerosis （SSc） patients have been developed. Regarding the treatment, all of the three types of PAH-targeted therapies including endothelin receptor antagonist, prostacyclin analogue and phosphodiesterase type-5 inhibitors have been showed effective for CTD associated PAH （CTD-APAH） by many randomized clinical trials and been recommended by the European treatment guidelines. The purpose of this review was to describe the clinical characteristics of CTD-APAH, the early detection methods and the approved pharmacological treatments, with a focus on the updated data associated with early detection and therapies.