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

Pulmonary arterial hypertension in systemic sclerosis

Benoît Lechartier  1 , Marc Humbert  2
Affiliations
Review

Pulmonary arterial hypertension in systemic sclerosis

Benoît Lechartier et al. Presse Med. 2021 Apr.

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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