The Cardiac Conundrum Recurrent Pericardial Tamponade in Limited Systemic Sclerosis a Case Report

Abstract Recurrent and symptomatic pericardial tamponade secondary to limited cutaneous systemic sclerosis (lcSSc) is an atypical presentation. This is a case of a 67-year-old female with a history of tobacco use, chronic GERD, and two decades of Raynaud’s phenomenon, who presented with increased dyspnea, fatigue, and pre-syncopal episodes. Clinical examination revealed low blood pressure, sclerodactyly, digital ulcers, calcinosis cutis, and telangiectasias. Diagnostic tests showed positive ANA at 1:2560 dilution, anti-centromere antibodies (>8), and normal anti-Scl-70, anti-dsDNA, anti-Smith, and anti-RNP antibodies, with normal complement levels and a negative antiphospholipid panel. A transthoracic echocardiogram (TTE) revealed a large pericardial effusion with tamponade physiology. Right heart catheterization (RHC) indicated normal filling pressures and pulmonary artery pressure. The patient underwent emergency pericardiocentesis, however 16 days later presented with recurrent tamponade, requiring balloon pericardiotomy, followed by a pericardial window. This case underscores the rarity and complexity of pericardial tamponade in lcSSc. Comprehensive evaluation ruled out common causes such as overlap syndrome, paraneoplastic syndrome processes, viral infections, hypothyroidism, and pulmonary hypertension. Pericardial tamponade in lcSSc is infrequent and suggests a poor prognosis with limited specific treatment options. Although lcSSc typically does not present manifest significant pericardial disease symptoms, this case highlights the need for prompt recognition and investigation of internal organ involvement.