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SYSTEMIC SCLEROSIS

Treatment of pulmonary hypertension in SSc

Nature Reviews Rheumatology volume 16, page 602 (2020)Cite this article

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In a multicentre study (n = 128), patients with systemic sclerosis (SSc) and both pulmonary hypertension and interstitial lung disease (SSc-PH-ILD) had a higher mortality rate than those with SSc and pulmonary arterial hypertension (SSc-PAH) (41.5% versus 21.4% at 3 years; P = 0.04). Following PAH-targeted therapy, improvement in WHO functional class was less frequent in patients with SSc-PH-ILD than in those with SSc-PAH (13.6% versus 33.3%, P = 0.02); haemodynamics improved similarly in both groups, despite the patients with SSc-PH-ILD having less severe haemodynamic impairment (lower mean pulmonary vascular resistance and carbon monoxide diffusion capacity) at baseline than those with SSc-PAH.

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    Sarah Onuora

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  1. Sarah Onuora
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Correspondence to Sarah Onuora.

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Onuora, S. Treatment of pulmonary hypertension in SSc. Nat Rev Rheumatol 16, 602 (2020). https://doi.org/10.1038/s41584-020-00512-y

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