Cardiopulmonary phenotype in systemic sclerosis associated pulmonary hypertension

Abstract

Introduction

Pulmonary hypertension (PH) associated with systemic sclerosis (SSc) increases morbidity and mortality. Cardiopulmonary comorbidities, as per the 2021 PH consensus, play a role in the choice of therapy between monotherapy and combination therapy.

Methods

A cross-sectional study was conducted in patients with SSc based on the 2013 ACR/EULAR criteria or very early disease (VEDOSS 2011). PH was considered if they met the following criteria: pulmonary artery systolic pressure (PASP) > 39 mmHg or peak tricuspid regurgitation velocity (PTRV) > 3.4 m/s, PASP between 33 and 39 mmHg or PTRV between 2.9 and 3.4 m/s plus two additional findings suggestive of PH. PH was classified as type 2 if LVEF < 50% or moderate to severe diastolic dysfunction was present; type 3 if extensive interstitial disease on tomography > 20% or forced vital capacity (FVC) < 75%; type 4 if abnormalities related to embolism were detected on scintigraphy or tomography. If patients did not meet these criteria, they were classified as type 1 PH. Complete data on cardiopulmonary risk factors and other factors were required. The frequency of these factors in the population and differences between groups based on risk factors were estimated.

Results

A total of 228 patients were selected. Three had type 2 PH, 24 had type 3, and 40 had type 1 PH, with the majority (75%) having at least one cardiopulmonary risk factor, and 47.5% having more than one. Mild diastolic dysfunction (25%) and hypertension (35%) were the most prevalent. In the type 1 PH group, those with risk factors experienced an increase in the number of years with Raynaud's phenomenon, anticentromere antibodies, and gastrointestinal symptoms (p < 0.05).

Conclusion

In patients with PH, 75% have one, and 45% have two or more risk factors.