Lausanne University Hospital, Department of Respiratory Medicine, Lausanne, Switzerland.
Université Paris-Saclay, Faculty of Medicine, 94270 Le Kremlin-Bicêtre, France; Hôpital Marie-Lannelongue, INSERM UMR_S 999 (Pulmonary Hypertension: Pathophysiology and Novel Therapies), Le Plessis-Robinson, France; Assistance publique-Hôpitaux de Paris (AP-HP), French Pulmonary Hypertension Reference Center, Hôpital Bicêtre, Department of Respiratory and Intensive Care Medicine, Le Kremlin-Bicêtre, France.
Presse Med. 2021 Apr;50(1):104062. doi: 10.1016/j.lpm.2021.104062. Epub 2021 Feb 3.
Pulmonary arterial hypertension (PAH) is a frequent and severe complication of systemic sclerosis (SSc) due to combined vasculopathy and fibrogenesis. Early diagnosis and treatment are highly challenging in SSc-PAH and require referral to an expert PAH centre. Diagnostic algorithms evolved in the last decade. Novel therapeutic options notably targeting pulmonary vascular remodeling are needed.
肺动脉高压（PAH）是系统性硬化症（SSc）的常见且严重的并发症，其发病机制与血管病变和纤维发生有关。SSc-PAH 的早期诊断和治疗极具挑战性，需要转诊至 PAH 专家中心。过去十年中已制定出诊断算法。目前需要新型治疗方法，尤其是针对肺血管重塑的治疗方法。
