CASE REPORT CREST Syndrome: A Rare Cause of Chronic Upper-Gastro Intestinal Haemorrhage in Adults
Abstract
ABSTRACT:
Background: CREST syndrome, a limited cutaneous variant of systemic sclerosis, rarely presents with chronic upper gastrointestinal haemorrhage. This case report explores the complex pathological mechanisms and management challenges of this uncommon manifestation.
Case Presentation: A 52-year-old female with established CREST syndrome presented with recurrent melena and hematemesis. Comprehensive clinical evaluation revealed extensive gastrointestinal telangiectasia with significant bleeding potential. Upper endoscopy demonstrated multiple fragile vascular lesions throughout the esophagus, stomach, and duodenum.The patient underwent detailed diagnostic imaging, including CT angiography and capsule endoscopy. Therapeutic interventions included argon plasma coagulation, tranexamic acid therapy, and systemic immunosuppression with mycophenolatemofetil.
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References
1. Adigun R, Goyal A, Hariz A. Systemic Sclerosis (Scleroderma) [Updated 2024 Apr 5]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2025 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK430875/
2. Tian J, Kang S, Zhang D, Huang Y, Zhao M, Gui X, et al. Global, regional, and national incidence and prevalence of systemic sclerosis. Clinical Immunology [Internet]. 2023 Mar [cited 2025 Jan 24];248:109267. Available from: https://linkinghub.elsevier.com/retrieve/pii/S1521661623000463
3. Sallam H, McNearney TA, Chen JD. Systematic review: pathophysiology and management of gastrointestinal dysmotility in systemic sclerosis (scleroderma). Aliment PharmacolTher. 2006;23(6):691-712. doi:10.1111/j.1365-2036.2006.02804.x
4. Crest syndrome: background, pathophysiology, etiology. 2024 Oct 29 [cited 2025 Jan 24]; Available from: https://emedicine.medscape.com/article/1064663-overview?form=fpf
5. Gabrielli A, Avvedimento EV, Krieg T. Scleroderma. N Engl J Med. 2009;360(19):1989-2003. doi:10.1056/NEJMra0806188
6. Allanore Y, Simms R, Distler O, et al. Systemic sclerosis. Nat Rev Dis Primers. 2015;1:15002. Published 2015 Apr 23. doi:10.1038/nrdp.2015.2
7. Petcu A, Ghib LJ, Grad SM, Popovici C, Rogojan L, Rednic NV, Rednic S. Upper gastrointestinal involvement in systemic sclerosis: Findings in a real-life setting. ExpTherMed. 2019 Dec;18(6):5095-5100. doi: 10.3892/etm.2019.8125. Epub 2019 Oct 24. PMID: 31798729; PMCID: PMC6880395.
8. Pătrîntașu DE, Sárközi HK, Lupușor E, Vlangăr IE, Rotariu GM, Rența IA, et al. A multidisciplinary approach as a goal for the management of complications in systemic scleroderma: a literature review and case scenario. Diagnostics [Internet]. 2023 Oct 28 [cited 2025 Jan 24];13(21):3332. Available from: https://www.mdpi.com/2075-4418/13/21/3332
9. Patnaik E, Lyons M, Tran K, Pattanaik D. Endothelial Dysfunction in Systemic Sclerosis. Int J Mol Sci. 2023 Sep 21;24(18):14385. doi: 10.3390/ijms241814385. PMID: 37762689; PMCID: PMC10531630.
10. Ren H, Liu L, Xiao Y, Shi Y, Zeng Z, Ding Y, et al. Further insight into systemic sclerosis from the vasculopathy perspective. Biomedicine & Pharmacotherapy [Internet]. 2023 Oct [cited 2025 Jan 24];166:115282. Available from: https://linkinghub.elsevier.com/retrieve/pii/S0753332223010739
11. McMahan ZH. Gastrointestinal involvement in systemic sclerosis: an update. CurrOpinRheumatol. 2019 Nov;31(6):561-568. doi: 10.1097/BOR.0000000000000645. PMID: 31389815; PMCID: PMC10266535.

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