Acute necrotizing pancreatitis as a manifestation of Behcet’s Disease: A case report and review of the literature

Background: Behçet’s disease (BD) is a chronic, relapsing, multisystemic vasculitis with varying frequency of gastrointestinal manifestations. Pancreatitis is a rare manifestation of BD. Case presentation: A 31-year-old man diagnosed with Behçet’s disease with a fiveyear history of recurrent orogenital ulcers, ocular involvement and pseudofolliculitis presented with severe epigastric pain radiating to back that worsens after meals and weight loss (10 kg in 3 months). He denied history of abdominal trauma, drug abuse or alcohol consumption. He was found to be hypertensive. Laboratory investigations revealed presence of leukocytosis, thrombocytosis, high inflammatory markers, low serum albumin, impaired renal functions, hyperglycemia and elevated serum amylase. Post contrast triphasic computed tomography scan showed a picture of necrotizing hemorrhagic pancreatitis. Bile and pancreatic ducts were normal. CT mesenteric angiography showed no evidence of aortic, celiac or superior mesenteric artery aneurysms, also splenic artery and veins were patent with no evidence of occlusion. After exclusion of other causes of acute pancreatitis, the condition has been attributed to BD. High dose of oral corticosteroids was started (60 mg/day) in addition to proper hydration, antihypertensive medications, intravenous antibiotics and insulin to control elevated blood sugar. Cystogastrostomy through endoscopic ultrasound was done to relieve the patient’s pain. Unfortunately, three months after the onset of pain, the patient developed systemic inflammatory response syndrome and passed away. Conclusion: Although acute pancreatitis is a rare gastrointestinal manifestation of BD, it is a possibly fatal one