Clinical Profile and Outcome of Pulmonary Hypertension With and Without Interstitial Lung Disease in Scleroderma

Abstract

Background: Scleroderma associated pulmonary arterial hypertension and interstitial lung disease (ILD) with pulmonary hypertension are considered to be separate pathological processes with different approach to management. In this study, we looked at the demographics, clinical features, and outcome of patients with both type of pulmonary hypertension in systemic sclerosis Methods: Patients diagnosed with scleroderma and with pulmonary hypertension as per transthoracic ECHO in the time period between 2010 and 2023 were retrospectively identified enrolled in the study. They were divided into two groups-isolated PAH and ILD-PH. ECHO parameters, clinical data were recorded Results: There were 800 patients with a diagnosis of systemic sclerosis during this period. In total, 49 incident scleroderma PH cases were identified (6.13%); 25 with diffuse cutaneous SSc (51.02%), 19 with limited cutaneous SSc (38.78%), and 5 with sine scleroderma (10.2%). Among the study group, 39 (79.59%) patients had ILD with PH and 10 (20.4%) had isolated PH (Table 1). Though the baseline ECHO parameters were similar in both the groups, features of severe PH - Right atrial and ventricular dilatation with Right ventricular dysfunction was more in Isolated PH than ILD-PH (50% vs 23%). Other clinical features were similar in both the groups (Table 2). Isolated PH was treated with vasodilatory therapy and all patients with ILD -PAH were treated with immunosuppression along with vasodilatory therapy. Follow up of ECHO parameters of the two groups at the end of 2 years showed better improvement in the ILD-PH group. Unsatisfactory long-term outcome as defined by clinical NYHA progression, hospitalization and need for long term oxygen therapy was similar in both isolated PH and PH-ILD (40% vs 41%). 1 year survival was 95.9% (2/49 lost to follow up). Conclusions: The prevalence of Pulmonary Hypertension in our scleroderma cohort of a tertiary care center was 6.13%. ILD-PH was 4 times more prevalent than isolated PH.46.94% of all our PH patients had it at presentation. The one-year survival of our cohort was 95.9% almost equivalent to other international cohorts