Article · Volume 15, Issue 4 · pp. 338–341

Diffuse Systemic Sclerosis with Interstitial Lung Disease and Severe Pulmonary Arterial Hypertension: A Case Report

Syed Zia Inamdar¹, Sushilkumar Londhe¹, Ravina Mehta¹, Sumanyu Katageri¹, Siddanagouda Biradar¹, Shashidhar Devaramani², Sharan Badiger²

¹Department of Pharmacy Practice, BLDEAs SSM College of Pharmacy and Research Centre, Vijayapura, Karnataka, INDIA.
²Department of Medicine, Shri B M Patil Medical College Hospital and Research Centre, Vijayapura, Karnataka, INDIA.

Abstract

Systemic sclerosis is a rare connective tissue disease categorized by extensive lung fibrosis, vascular and immunologic abnormalities. Autoimmune antibodies like anticentromere, anti-Scl-70 (anti-topoisomerase I), and anti-RNA polymerase II are commonly involved in progressive disease. Clinical presentations consist of Raynaud’s phenomenon, digital ulcers; pericardial effusion, and telangiectasia and are mostly associated with pulmonary complications like interstitial lung disease and pulmonary arterial hypertension. Disease-specific therapy is unavailable instead; symptomatic management is only the line of treatment. We report here a case of diffuse systemic sclerosis with interstitial lung disease and pulmonary arterial hypertension.

Keywords: Diffuse systemic sclerosis; Interstitial lung disease; Pulmonary arterial hypertension; Raynaud’s phenomenon

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