Introduction
Systemic sclerosis (SSc), also known as scleroderma, is a complex autoimmune connective tissue disorder characterized by progressive fibrosis of the skin and internal organs. CREST syndrome, a limited cutaneous variant of systemic sclerosis, presents unique clinical manifestations that can lead to significant complications, including gastrointestinal involvement [1]. While the global SSc incidence and newlydiagnosedpopulation were estimated to be 8.64 per 100,000 person-years (1.78–23.57) and0.67 million (0.14–1.84)people annually, respectively, gastrointestinal bleeding remains an uncommon yet potentially life-threatening manifestation [2].
Chronic upper gastrointestinal haemorrhage in CREST syndrome patients can result from various pathological mechanisms, including mucosal atrophy, telangiectasia, and vascular fragility [3]. These complications arise from the underlying autoimmune process that causes widespread microvascular damage and fibrotic changes in the gastrointestinal tract [4]. Despite the recognized potential for gastrointestinal involvement, chronic upper gastrointestinal haemorrhage as a primary presentation remains a rare clinical scenario that challenges diagnostic & management strategies [5].
This case report aims to highlight the complex interplay between CREST syndrome and chronic upper gastrointestinal haemorrhage, contributing to the understanding of this unusual clinical manifestation and potentially guiding future diagnostic and therapeutic approaches.
Case Report
A 52-year-old female presented to our General Medicine OPD with a two-year history of intermittent melena and recurrent episodes of hematemesis. Her medical history was remarkable for CREST syndrome, diagnosed six years prior, characterized by calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia.
Physical examination revealed characteristic skin thickening of the hands and face, with multiple telangiectatic lesions on the lips and fingers. Laboratory investigations showed mild anaemia with hemoglobin of 9.4 g/dL, consistent with chronic blood loss.
Upper endoscopy demonstrated extensive mucosal telangiectasia throughout the esophagus, stomach, and duodenum, with multiple fragile vascular lesions prone to spontaneous bleeding.
Detailed imaging, including CT angiography and capsule endoscopy, confirmed widespread gastrointestinal microvasculature abnormalities characteristic of systemic sclerosis. The patient underwent multiple therapeutic interventions, including argon plasma coagulation and tranexamic acid therapy to manage chronic haemorrhage.
Over a six-month follow-up period, the patient received periodic transfusions and underwent aggressive management of her gastrointestinal vascular lesions. Immunosuppressive therapy with mycophenolate mofetil was initiated to control underlying autoimmune processes and potentially reduce vascular fragility.
The case highlights the complex management challenges of gastrointestinal complications in CREST syndrome, demonstrating the critical importance of a multidisciplinary approach involving rheumatology, gastroenterology, and interventional specialists in managing this rare manifestation of systemic sclerosis.
Discussion
The presented case illustrates the complex gastrointestinal manifestations of CREST syndrome, particularly focusing on chronic upper gastrointestinal haemorrhage. Our findings align with previous literature documenting the significant vascular pathology associated with systemic sclerosis, characterized by widespread telangiectasia and microvascular abnormalities [6].
Petcu A et al. reported similar endoscopic findings in a comprehensive study of 79 scleroderma patients, noting that gastrointestinal involvement occurs in up to 72.1% of cases, with significant hemorrhagic potential [7]. Our case demonstrates the extreme end of this spectrum, where chronic bleeding becomes the predominant clinical manifestation, a scenario reported in less than 5% of systemic sclerosis cases.
The management approach in our case, combining argon plasma coagulation and systemic immunosuppression, mirrors recommendations by Sallam and McNearney,
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