Abstract

Introduction: One of the most challenging aspects of systemic sclerosis is the potential involvement of the cardiopulmonary system, which can lead to significant morbidity and mortality if not detected and managed promptly. Aim: To assess the clinical characteristics and prevalence of specific cardiopulmonary involvement and to investigate its association with disease duration in patients with systemic sclerosis from South India. Materials and Methods: This cross-sectional, observational study was conducted at the Department of General Medicine, Government Medical College, Thrissur, India, between January 1, 2020, and December 31, 2020. Forty patients with systemic sclerosis were included according to the European Alliance of Associations for Rheumatology (EULAR) and the American College of Rheumatology classification criteria. Skin thickening was evaluated using the modified Rodnan Skin Score (mRSS). Investigations such as chest X-ray, echocardiogram, and antibody profiling were conducted to evaluate cardiopulmonary involvement. The data were analysed using Statistical Package for Social Sciences (SPSS) version 23.0, and the Chi-square test was used to compare qualitative variables between groups. Results: Sixteen patients (40%) had an mRSS score between 11 and 20, followed by 14 patients (35%) with mRSS scores between 21 and 30. The prevalence of Interstitial Lung Disease (ILD) was 50%. The echocardiogram findings showed mitral regurgitation and moderate Pulmonary Arterial Hypertension (PAH) in 30% (n=12) and 25% (n=10) of patients, respectively. High-Resolution Computed Tomography (HRCT) and pulmonary function tests were normal in 10 patients (25%). The duration of the disease was three to eight years in 14 patients and eight to 12 years in 12 patients. Both ILD and PAH were significantly associated with the duration of the disease (p<0.05). Antiscleroderma-70 and anti-centromere were the most common antibodies present in patients with ILD and PAH, respectively. Conclusion: The study indicated a high prevalence of PAH and ILD in patients with systemic sclerosis. Moreover, both ILD and PAH were significantly associated with the duration of the underlying disease, with longer disease duration being associated with a higher likelihood of these conditions.

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