Echoes of Scleroderma: A Case Report Unmasking CREST (Calcinosis, Raynaud's Phenomenon, Oesophageal Dysmotility, Sclerodactyly, and Telangiectasia) Syndrome With Point-of-Care Ultrasound in Acute Medicine
Abstract
CREST syndrome, a limited form of systemic sclerosis (SSc), is characterised by calcinosis, Raynaud's phenomenon, oesophageal dysmotility, sclerodactyly, and telangiectasia. It is an autoimmune disorder marked by connective tissue fibrosis and vasculopathy, with pulmonary arterial hypertension (PAH) being one of its most severe complications. PAH has a poor prognosis and often presents with nonspecific symptoms, which can delay diagnosis and treatment. We present the case of a 54-year-old woman with progressive shortness of breath and bilateral leg swelling, initially managed as possible heart failure. Imaging ruled out pulmonary embolism, but further review revealed oesophageal fluid. Clinical examination showed peri-oral puckering, telangiectasia, calcinosis, and sclerodactyly, raising the suspicion of CREST syndrome. Bedside echocardiography suggested pulmonary hypertension, while serology confirmed strongly positive antinuclear and anticentromere antibodies. She was referred to rheumatology and cardiology, with plans for mycophenolate mofetil initiation and further PAH-focused management. This case highlights the diagnostic challenges associated with CREST syndrome and emphasises the importance of thorough clinical assessment, imaging review, and antibody testing. Early identification of SSc and its complications allows for prompt intervention, multidisciplinary management, and better patient outcomes in conditions that typically have a poor prognosis.
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