Management of pulmonary arterial hypertension in systemic sclerosis: from classical treatments to new horizons - PubMed

2 days ago

Pulmonary arterial hypertension (PAH) is a severe disease characterized by progressive thickening and obliteration of pulmonary vessels, leading to increased vascular resistance, elevated pulmonary artery pressures, and right heart failure.
Systemic sclerosis (SSc) is the most common condition associated with PAH in Western countries, with SSc-PAH presenting a more aggressive clinical course, poorer response to conventional therapies, and worse prognosis compared to other forms of PAH.
Despite similarities in management between SSc-PAH and idiopathic PAH, there is a crucial need for treatment strategies specifically tailored to SSc-PAH.
A systematic literature review assessed the evidence on SSc-PAH management, covering conventional therapies, immunosuppressants, nonconventional drugs, and surgical/interventional procedures.
Key gaps identified include the lack of studies focusing on SSc-PAH, heterogeneity in hemodynamic severity (with scarce data for mild PAH), and exclusion of relevant comorbidities like interstitial lung disease.
Pragmatic, experience-based suggestions are provided to help clinicians manage SSc-PAH, especially in cases where robust data are lacking.

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