Fatal Cardiac Tamponade in Newly Diagnosed Systemic Sclerosis with Early Cutaneous Disease: A Case Report

Background:Systemic Sclerosis (SSc) is a rare systemic autoimmune disease with a potential to affect most organ systems of the body. Symptomatic pericardial effusion is rarely reported as the first reason for hospital visit in a case of undiagnosed SSc. Case summary: A 53year old Nigerian woman was admitted with dyspnea, recurrent Raynaud’s phenomenon and scanty skin sclerosis over the dorsum of the hands and the upper back. Echocardiography revealed moderate pericardial effusion and severe pulmonary hypertension. She had positive antinuclear antibody and anti-Scl-70. A diagnosis of systemic sclerosis was made and she was treated with low dose prednisolone, frusemide and tadalafil. She recovered with complete resolution of the pleural effusion over 2 weeks but she presented to the emergency unit a month later in cardiac tamponade. Repeat echocardiography showed re-accumulated pericardial effusion with a compressed left ventricle. She suffered a cardiac arrest before a pericardiocentesis could be done and she could not be revived. Conclusion: Early SSc should be considered while investigating a case of pericardial effusion of unidentified cause. These cases may be more prone to tamponade even before a large amount of pericardial effusion accumulates. Keywords: Systemic sclerosis; Pericarditis; Cardiac tamponade; Raynaud’s disease; Antinuclear antibody