Pulmonary Hypertension: How to Best Treat the Different Scleroderma Phenotypes?

Benjamin D Korman; Daniel J Lachant; Flavia V Castelino

doi:10.1016/j.rdc.2023.01.015

Pulmonary Hypertension: How to Best Treat the Different Scleroderma Phenotypes?

Rheum Dis Clin North Am. 2023 May;49(2):345-357. doi: 10.1016/j.rdc.2023.01.015.

Authors

Benjamin D Korman¹, Daniel J Lachant², Flavia V Castelino³

Affiliations

¹ Division of Allergy, Immunology, and Rheumatology, University of Rochester Medical Center, 601 Elmwood Avenue, Box 695, Rochester, NY 14642, USA. Electronic address: benjamin_korman@urmc.rochester.edu.
² Division of Pulmonary and Critical Care Medicine, University of Rochester Medical Center, 601 Elmwood Avenue, Box 692, Rochester, NY 14642, USA.
³ Division of Rheumatology, Massachusetts General Hospital, 55 Fruit Street, Yawkey 4B, Boston, MA 02114, USA.

PMID: 37028839
DOI: 10.1016/j.rdc.2023.01.015

Abstract

Pulmonary hypertension (PH) is a leading cause of morbidity and mortality in systemic sclerosis (SSc). PH is a heterogenous condition and several different forms of PH are associated with SSc, including pulmonary arterial hypertension (PAH) resulting from a pulmonary arterial vasculopathy, PH due to interstitial lung disease, PH due to left heart disease, and PH due to thromboembolic disease. Extensive research has led to an improved understanding of the mediators involved in the pathogenesis of SSc-PH. Initial combination therapy is the preferred treatment approach for SSc-PAH and requires coordinated care with a multidisciplinary team including rheumatology, pulmonology, and cardiology.

Keywords: Pulmonary hypertension; Scleroderma; Screening; Systemic sclerosis; Treatment.

Publication types

Review
Research Support, Non-U.S. Gov't

MeSH terms

Humans
Hypertension, Pulmonary* / etiology
Hypertension, Pulmonary* / therapy
Phenotype
Scleroderma, Systemic* / complications
Scleroderma, Systemic* / therapy