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Systemic Sclerosis Sine Scleroderma Presenting as Pulmonary Arterial Hypertension - A Case Report

Ali Karar

Journal of Clinical Rheumatology and Immunology, 2024

Abstract

A 60-year-old married male farmer with a significant medical history, including STElevation Myocardial Infarction (STEMI), coronary artery bypass grafting (CABG), and Raynaud’s disease, presented to the emergency department with sudden-onset syncope and progressive shortness of breath on exertion. The syncope episode involved an abrupt loss of consciousness, with spontaneous recovery and no associated symptoms like jerky movements or tongue biting. The shortness of breath had been gradually worsening over several weeks, becoming noticeable even during minimal exertion. On examination, the patient appeared chronically ill but had stable vital signs cardiovascular examination revealed bilateral pedal edema, while respiratory assessment and Neurological evaluation were normal. Blood tests indicated normal full blood count (FBC) and urea and electrolytes (U+E), but slight elevations in CRP 29 mg/L and ESR 20 mm/hr. Troponin levels remained stable, but brain natriuretic peptide (BNP) was markedly elevated at 2061 pg/mL. The electrocardiogram (ECG) showed right axis deviation and incomplete right bundle branch block (RBBB). A CT scan of the brain ruled out any acute pathology, such as stroke or hemorrhage. A previous echocardiogram (ECHO) had shown a left ventricular ejection fraction (LVEF) of 50-55%, indicating good left ventricular function. Given the findings, the patient was started on Furosemide 40 mg daily and advised to follow a fluid restriction regimen to prevent…

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