Pulmonary arterial hypertension in systemic sclerosis - 10/03/21

Doi : 10.1016/j.lpm.2021.104062

Benoît Lechartier ^a, Marc Humbert ^b,^c,^d,⁎
^a Lausanne University Hospital, Department of Respiratory Medicine, Lausanne, Switzerland
^b Université Paris-Saclay, Faculty of Medicine, 94270 Le Kremlin-Bicêtre, France
^c Hôpital Marie-Lannelongue, INSERM UMR_S 999 (Pulmonary Hypertension: Pathophysiology and Novel Therapies), Le Plessis-Robinson, France
^d 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

^⁎Corresponding author at: Université Paris-Saclay, Faculty of Medicine, 94270 Le Kremlin-Bicêtre, France.Université Paris-Saclay, Faculty of MedicineLe Kremlin-Bicêtre94270France

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Abstract

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.

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