Autoimmune pancreatitis associated with Sjögren's syndrome: A case report

Autoimmune pancreatitis (AIP) is a rare but increasingly recognized entity. It is characterized by diverse diagnostic criteria, including pancreatic and extrapancreatic involvement, immunological abnormalities, and a therapeutic response to corticosteroids. 

We report the case of Mrs. R.E.B, a 68-year-old woman followed for decompensated cirrhosis of autoimmune origin, specifically primary biliary cirrhosis, treated with ursodeoxycholic acid. She was admitted for acute epigastric pain associated with vomiting. The diagnosis of acute pancreatitis was made based on clinical presentation, elevated lipase levels, and abdominal CT findings consistent with pancreatitis. As part of the etiological workup, an abdominal ultrasound and a metabolic phospholipid profile were performed, both of which were negative. The investigation of an autoimmune etiology led to a workup including IgG4 levels, which were found to be three times the normal value. This was followed by MR cholangiopancreatography (MRCP), which revealed a “sausage-shaped” pancreas, supporting the diagnosis of autoimmune pancreatitis. The search for other systemic involvement revealed the presence of xerophthalmia and xerostomia upon questioning—symptoms the patient had previously overlooked. A labial salivary gland biopsy confirmed lymphocytic sialadenitis, consistent with Sjögren’s syndrome. The suggestive findings on MRI, positive serology, and the association with Sjögren’s syndrome led to the diagnosis of autoimmune pancreatitis (AIP). Corticosteroid therapy was initiated alongside symptomatic treatment for acute pancreatitis, leading to a remarkable clinical and biological improvement.