Pulmonary arterial hypertension in systemic sclerosis

Benoît Lechartier; Marc Humbert

doi:10.1016/j.lpm.2021.104062

Pulmonary arterial hypertension in systemic sclerosis

Presse Med. 2021 Apr;50(1):104062. doi: 10.1016/j.lpm.2021.104062. Epub 2021 Feb 3.

Authors

Benoît Lechartier¹, Marc Humbert²

Affiliations

¹ Lausanne University Hospital, Department of Respiratory Medicine, Lausanne, Switzerland.
² 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. Electronic address: marc.humbert@aphp.fr.

PMID: 33548377
DOI: 10.1016/j.lpm.2021.104062

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.

Publication types

Review

MeSH terms

Algorithms
Biomarkers / blood
Cardiac Catheterization / methods
Early Diagnosis
Echocardiography, Doppler
Endomyocardial Fibrosis
Exercise Test
Humans
Lung Transplantation
Pulmonary Arterial Hypertension / diagnosis
Pulmonary Arterial Hypertension / etiology*
Pulmonary Arterial Hypertension / physiopathology
Pulmonary Arterial Hypertension / therapy
Pulmonary Gas Exchange
Referral and Consultation
Scleroderma, Systemic / complications*

Substances

Biomarkers