Background/Purpose: Systemic sclerosis (SS) is a complex autoimmune rheumatic disease characterized by immune-mediated activation of both innate and adaptive immunity. Pulmonary involvement, specifically pulmonary arterial hypertension (PAH), is the leading cause of death in SS. Cardiac involvement, although mostly asymptomatic, has been linked to poor prognosis and increased mortality.The purpose of the study is to investigate the association of PAH as a risk factor for developing cardiovascular complications in individuals with SS.

Methods: National Inpatient Sample Database was used to procure individuals with SS, and patients were divided into two groups based on the presence of PAH. Baseline demographics and comorbidities were collected using ICD-10 codes. Patients with missing data, age under 18, or those diagnosed with pulmonary hypertension due to another etiology were excluded. Greedy propensity matching using R was performed to match patients with and without PAH on age, race, gender, and 15 other comorbidities, including anticoagulation use. Univariate analysis pre- and post-match were performed. Binary logistic regression was performed post-match to assess whether PAH was an independent risk factor for cardiovascular complications. A p-value of < 0.05 was considered statistically significant.

Results: Out of 17,675 patients included in the study, 1,294 had PAH. PAH patients were older and had a higher prevalence of baseline comorbidities. On univariate analysis, PAH patients had significantly higher acute heart failure (29.8% vs. 17.9%), tachyarrhythmias (27.7% vs. 18.5%), conduction disease (6.9% vs. 4.2%), aortic valve disease (5.9% vs 3.5%) and tricuspid valve disease (1.9% vs 0.7%). After matching, binary logistic regression analysis showed PH patients having higher conduction disease (OR: 1.73 [1.22-2.45]; p< 0.01) and tachyarrhythmias (OR: 1.24 [1.04-1.48]; p=0.02). PAH in SS patients was associated with a higher prevalence of conduction disorders and tachyarrhythmias.

Conclusion: The probable etiology of these findings can be attributed to microvascular dysfunction and myocardial fibrosis commonly associated with SS. Regular cardiovascular monitoring with a lower threshold for cardiac workup is recommended for SS patients, particularly after the emergence of PAH. Large-scale trials with a specific focus on SS patients with PAH are required to formulate appropriate preventive and management guidelines.

« Back to ACR Convergence 2024

ACR Meeting Abstracts - https://acrabstracts.org/abstract/exploring-the-cardiovascular-complications-of-pulmonary-arterial-hypertension-in-individuals-with-systemic-sclerosis/