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Introduction: Pulmonary hypertension (PH) is a leading cause of mortality in systemic sclerosis (SSc), yet data from India remain limited. Although right heart catheterisation (RHC) is the gold standard, restricted access necessitates reliance on transthoracic echocardiography (TTE) for diagnosis and follow-up. This study describes the clinical characteristics and echocardiographic features of SSc-associated PH (SSc-PH) and compares isolated PH with PH associated with interstitial lung disease (PH-ILD). Methodology: In this retrospective study, patients fulfilling the American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) criteria for SSc and diagnosed with moderate- to high probability PH (as per European Society of Cardiology/European Respiratory Society ESC/ERS criteria) on TTE between January 2006 and January 2023 were included. Patients were categorised into isolated PH or PH-ILD groups. Clinical worsening was defined as the need for long-term oxygen therapy (LTOT), hospitalisation or worsening New York Heart Association (NYHA) functional class. Results: Forty-nine patients met the inclusion criteria, of whom 79.6% were female. Thirty-nine patients had PH-ILD and 10 had isolated PH. Anti-centromere (CENP) antibody positivity was significantly higher in isolated PH. Clinical manifestations were broadly comparable between groups. Echocardiographic markers of severe PH, including right ventricular (RV) dysfunction and right heart dilatation, were more frequent in isolated PH. Serial echocardiography demonstrated improvement in tricuspid regurgitation velocity (TRV), pulmonary acceleration time (PAT) and right heart dimensions in both groups, with greater improvement observed in the PH-ILD group. Rates of clinical worsening were similar, but baseline pericardial effusion was significantly associated with worsening. Conclusion: Overall, isolated PH and PH-ILD showed comparable clinical profiles and outcomes, highlighting the utility of echocardiography as a practical diagnostic and monitoring tool for SSc-PH in resource-limited settings.
Jacob et al. (Wed,) studied this question.