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

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), anti-RNA polymerase II are commonly involved in progressive disease. Clinical presentations consist of Raynaud’s phenomenon, digital ulcers; pericardial effusion, telangiectasia mostly associated with pulmonary complications interstitial arterial hypertension. Disease-specific therapy unavailable instead; symptomatic management only the line treatment. We report here case diffuse systemic Keywords: Diffuse sclerosis, Interstitial disease, Pulmonary hypertension, phenomenon.