Systemic sclerosis is a multisystem connective tissue disease that is associated with substantial morbidity and mortality. Visceral organ involvement is common in patients with systemic sclerosis and occurs independently of skin manifestations. Pulmonary hypertension (PH) is an important and prevalent complication of systemic sclerosis. The clinical classification of PH cohorts conditions with similar pathophysiological mechanisms into one of five groups. While patients with systemic sclerosis can manifest with a spectrum of pulmonary vascular disease, notable clinical groups include group 1 pulmonary arterial hypertension (PAH) associated with connective tissues disease, PAH with features of capillary/venous involvement, group 2 PH associated with left heart disease, and group 3 PH associated with interstitial lung disease. Considerable efforts have been made to advance screening methods for PH in systemic sclerosis including the DETECT and ASIG (Australian Scleroderma Interest Group) composite algorithms. Current guidelines recommend annual assessment of the risk of PAH as early recognition may result in attenuated hemodynamic impairment and improved survival. The treatment of PAH associated with systemic sclerosis requires a multidisciplinary team including a PH specialist and a rheumatologist to optimize immunomodulatory and PAH-specific therapies. Several potential biomarkers have been identified and there are several promising PAH therapies on the horizon such as the novel fusion protein sotatercept. This chapter provides an overview of PH in systemic sclerosis, with a specific focus on group 1 PAH.

系统性硬化症是一种多系统结缔组织疾病，与大量发病率和死亡率相关。内脏器官受累在系统性硬化症患者中很常见，并且与皮肤表现无关。肺动脉高压（PH）是系统性硬化症的重要且普遍的并发症。 PH 队列的临床分类将具有相似病理生理机制的情况分为五组之一。虽然系统性硬化症患者可表现为一系列肺血管疾病，但值得注意的临床分组包括与结缔组织疾病相关的第 1 组肺动脉高压 (PAH)、具有毛细血管/静脉受累特征的 PAH、与左心疾病相关的第 2 组 PH ，以及与间质性肺疾病相关的第 3 组 PH。人们为推进系统性硬化症 PH 筛查方法做出了大量努力，包括 DETECT 和 ASIG（澳大利亚硬皮病兴趣小组）复合算法。目前的指南建议每年对 PAH 风险进行评估，因为早期识别可能会减轻血流动力学损伤并提高生存率。与系统性硬化症相关的 PAH 的治疗需要包括 PH 专家和风湿病专家在内的多学科团队来优化免疫调节和 PAH 特异性治疗。几种潜在的生物标志物已经被鉴定出来，并且有几种有前途的 PAH 疗法即将出现，例如新型融合蛋白 sotatercept。本章概述了系统性硬化症中的 PH，特别关注 1 类 PAH。