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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

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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