Pulmonary hypertension (PH) commonly affects patients with systemic sclerosis (SSc) and is associated with significant morbidity and increased mortality. PH is a heterogenous condition and several different forms can be associated with SSc, including pulmonary arterial hypertension (PAH) resulting from a pulmonary arterial vasculopathy, PH due to left heart disease and PH due to interstitial lung disease. The incidence of pulmonary veno-occlusive disease is also increased. Accurate and early diagnosis to allow optimal treatment is, therefore, essential. Recent changes to diagnostic haemodynamic criteria at the 6th World Symposium on Pulmonary Hypertension have resulted in therapeutic uncertainty regarding patients with borderline pulmonary haemodynamics. Furthermore, the optimal pulmonary vascular resistance threshold for diagnosing PAH and the role of exercise in identifying early disease require further elucidation. In this article we review the epidemiology, diagnosis, outcomes and treatment of the spectrum of pulmonary vascular phenotypes associated with SSc.

肺动脉高压 (PH) 通常会影响系统性硬化症 (SSc) 患者，并且与显着的发病率和死亡率增加有关。PH 是一种异质性疾病，几种不同的形式可能与 SSc 相关，包括肺动脉血管病变引起的肺动脉高压 (PAH)、左心疾病引起的 PH 和间质性肺病引起的 PH。肺静脉闭塞病的发病率也增加。因此，准确和早期诊断以实现最佳治疗至关重要。最近在第六届世界肺动脉高压研讨会上对诊断血流动力学标准的修改导致了对临界肺血流动力学患者的治疗不确定性。此外，诊断 PAH 的最佳肺血管阻力阈值和运动在识别早期疾病中的作用需要进一步阐明。在这篇文章中，我们回顾了与 SSc 相关的一系列肺血管表型的流行病学、诊断、结果和治疗。