Clinical and Serological Profile of Systemic Sclerosis Patients in a Tertiary Care Center in Kashmir, North India

Abstract

Abstract Background: Systemic sclerosis (SSc) is an autoimmune disease that varies by ethnicity and geography. India, being a multiethnic country with many different geographical regions, could have such differences. However, due to a lack of data, such differences remain unknown. Aim: The aim of the study was to study clinical manifestations, organ involvement, autoantibodies, and associated autoimmune diseases in subtypes of SSc patients from Jammu and Kashmir. Materials and Methods: This single-center observational study was conducted in two parts, retrospective and prospective. A total of 112 patients were included. Data were compiled from inpatient and outpatient department records of 66 patients for retrospective part from 2012 to 2016. Prospective data were taken from 46 patients from 2016 to 2018. Patient history, clinical examination, pulmonary function test, high-resolution computed tomography (HRCT), and echocardiography findings were recorded. Results: The female: male ratio was 15:1, with a mean age of 38.3 ± 12.51 years. Sixty-five (58%) patients had limited cutaneous SSc (LcSSc), 22 (19.6%) had diffuse cutaneous SSc, 5 (4.4%) had early scleroderma, and 20 (17.8%) patients had overlap with other autoimmune diseases. Antinuclear antibody was present in 87.5% of patients. Interstitial lung disease was seen in 80.41% of the patients by HRCT, with majority having nonspecific interstitial pneumonitis pattern. Pulmonary artery hypertension was present in 14.28% of the study patients. Conclusions: In the current study and the rest of India, disease onset is a decade earlier than in the Caucasian population. There is a significant variation in presentation among patients in India, with patients from Jammu and Kashmir reporting a higher prevalence of Raynaud’s phenomenon and LcSSc.