Necrotising pancreatitis in a patient with CREST syndrome: a rare autoimmune gastrointestinal complication

Authors

• Antonio Al Hazzouri Department of Internal Medicine, University of Balamand, Beirut, Lebanon
• Philippe Attieh Department of General surgery, University of Balamand, Beirut, Lebanon
• George Ghaleb Faculty of Medicine and Medical Sciences, University of Balamand, Beirut, Lebanon
• Ahmad Khalil Faculty of Medicine and Medical Sciences, University of Balamand, Beirut, Lebanon
• Noura Chamchoum Faculty of Medicine and Medical Sciences, University of Balamand, Beirut, Lebanon
• Karam Karam Department of Hepatology and Gastroenterology, University of Balamand, Beirut, Lebanon
• Elias Fiani Department of Hepatology and Gastroenterology, University of Balamand, Beirut, Lebanon

elias.fiani@hotmail.com

Keywords

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.

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