Pulmonary Hypertension and Arrhythmogenic Right Ventricular Cardiomyopathy in Atypical Sjögren’s Syndrome: A Rare Case Report

Sjögren’s syndrome is a chronic autoimmune condition in which the majority of patients present with sicca symptoms. About 20% of patients, however, do not have sicca symptoms and instead present predominantly with systemic or extraglandular involvement, posing a diagnostic challenge. Pulmonary Hypertension (PH) manifests as elevated pulmonary arterial pressure due to remodelling of the pulmonary vasculature as a result of various factors, including systemic involvement, infectious agents, connective tissue diseases, or toxin/drug exposure. PH in Sjögren’s syndrome is a dangerous complication that can significantly affect patient outcomes. Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) is a genetic cardiomyopathy characterised by fibrofatty replacement of the right ventricular myocardium, predisposing patients to arrhythmias which may cause abrupt collapse or sudden death. Sjögren’s syndrome and ARVC are two distinct disorders that may lead to grave outcomes when they co-exist. Present case is a rare case of atypical Sjögren’s syndrome with PH and ARVC in a patient who suffered a cardiac arrest during her initial evaluation. Further work-up revealed ARVC, following which appropriate treatment was initiated. This case underlines the need for clinicians to consider Sjögren’s syndrome even in the absence of sicca symptoms, especially when unexplained systemic or cardiovascular findings are present. The co-existence of ARVC added further diagnostic complexity. Overlapping pathophysiology— right ventricular strain from PH combined with intrinsic ARVC-related myocardial damage—may accelerate clinical deterioration.