Evidence map›Paper›PMID 41229628›Full record

ArticleEuropean journal of case reports in internal medicine2025

Necrotising Pancreatitis in a Patient with Crest Syndrome: A Rare Autoimmune Gastrointestinal Complication.

Antonio Al Hazzouri, Philippe Attieh, George Ghaleb, Ahmad Khalil, Noura Chamchoum, Karam Karam, Elias Fiani

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Article in European journal of case reports in internal medicine, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.

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1 · What the graph read from it

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4 · The record

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5 · Who and what money

Authors and funding

7 authors.

Antonio Al HazzouriDepartment of Internal Medicine, University of Balamand, Beirut, Lebanon.
Philippe AttiehDepartment of General surgery, University of Balamand, Beirut, Lebanon.
George GhalebFaculty of Medicine and Medical Sciences, University of Balamand, Beirut, Lebanon.
Ahmad KhalilFaculty of Medicine and Medical Sciences, University of Balamand, Beirut, Lebanon.
Noura ChamchoumFaculty of Medicine and Medical Sciences, University of Balamand, Beirut, Lebanon.
Karam KaramDepartment of Hepatology and Gastroenterology, University of Balamand, Beirut, Lebanon.
Elias FianiDepartment of Hepatology and Gastroenterology, University of Balamand, Beirut, Lebanon.

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

Necrotising pancreatitis is a severe and life-threatening form of acute pancreatitis characterised by the necrosis of pancreatic and peripancreatic tissues due to intense inflammation and autodigestion. While the condition is uncommon, its association with autoimmune diseases such as systemic sclerosis - particularly the limited form known as CREST syndrome - is even rarer and underreported. We present a case of a 55-year-old male with a known diagnosis of CREST syndrome who was admitted with severe epigastric pain radiating to the back. Laboratory tests revealed markedly elevated lipase levels, while imaging with a CT scan showed extensive peripancreatic and retroperitoneal fluid collections consistent with necrotising pancreatitis. A thorough workup excluded common causes such as gallstones, alcohol use, hypertriglyceridemia, viral infections and drug-induced pancreatitis. Consequently, the pancreatitis was attributed to the patient's underlying autoimmune condition. The patient was managed with aggressive intravenous hydration, pain control and bowel rest, followed by gradual reintroduction of oral intake. He showed rapid clinical improvement and was discharged in a stable condition. This case underscores the importance of recognising rare gastrointestinal complications of systemic sclerosis. Vascular damage, immune dysregulation and fibrosis associated with CREST syndrome likely contributed to pancreatic ischaemia and necrosis. Clinicians should maintain a high index of suspicion for atypical presentations of pancreatitis in autoimmune patients, especially when routine aetiologies are excluded. A multidisciplinary approach is essential to ensure timely diagnosis and optimal management. This report highlights the need for increased awareness and further research into the gastrointestinal manifestations of systemic sclerosis, including its rare but significant association with necrotising pancreatitis. LEARNING POINTS: This case illustrates an unusual overlap between CREST syndrome and necrotizing pancreatitis, a severe condition associated with high morbidity and mortality.

Indexed as

autoimmune pancreatitisCREST syndromenecrotising pancreatitis

Identifiers

PMID41229628
PMCPMC12604894

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