Case Report Vanessa C Costilla, MD,1
Another Great Mimicker—Autoimmune Pancreatitis Rahul Pannala, MD,1
Cuong C Nguyen, MD,1 Joseph M Collins, MD2 and G Anton Decker, MBBCh, MRCP, MHA1 1. Pancreas Clinic, Division of Gastroenterology; 2. Department of Radiology, Mayo Clinic Arizona
M Edwyn Harrison, MD,1
Abstract
A 52-year-old man presented with acute pancreatitis and a malignant-looking distal common bile duct stricture on magnetic resonance cholangiopancreatography and endoscopic retrograde cholangiopancreatography. He was referred for pancreaticoduodenectomy, but requested a second opinion. A positron emission tomography (PET) scan demonstrated numerous areas of hypermetabolism, including the submandibular glands. A submandibular gland biopsy confirmed the diagnosis of multi-organ involvement by immunoglobulin G4-associated systemic disease (ISD) and he was effectively treated with immunosuppressive therapy. This case highlights the diverse clinical spectrum of autoimmune pancreatitis (AIP) and ISD, and the clinical challenge in differentiating this condition from either pancreatic or cholangiocarcinoma. The clinical features, diagnosis, and management of AIP and ISD are discussed.
Keywords Immunoglobulin 4, autoimmune pancreatitis, immunoglobulin 4-associated systemic disease
Disclosure: The authors have no conflicts of interest to declare. Received: March 9, 2011 Accepted: May 31, 2011 Citation: US Gastroenterology & Hepatology Review, 2011;7(1):68–71 Correspondence: G Anton Decker, MBBCh, MRCP, MHA, Associate Professor of Medicine, Division of Gastroenterology and Hepatology, Mayo Clinic, 13400 East Shea Boulevard, Scottsdale, AZ 85259. E:
decker.anton@
mayo.edu
A 52-year-old man with a history of gastroesophageal reflux disease, hypertension, hyperlipidemia, and asthma presented to an Emergency Department with dull epigastric pain. He had no history of alcohol, tobacco, or illicit drug use. On examination he was found to be jaundiced and had mild epigastric tenderness. Laboratory studies were significant for a lipase of 1,305U/l (reference range [RR] 7–60) and amylase of 495U/l (RR 26–102). Liver enzymes were elevated with an aspartate transaminase (AST) and alanine transaminase (ALT) of 658IU/l (RR 8–48) and 533IU/l (RR 7–55), respectively. Bilirubin was also elevated at 2.8mg/dl (RR 0.1–1.1). Carbohydrate antigen 19-9 was not elevated at 37U/ml (RR <55). An abdominal ultrasound and computed tomography (CT) scan were negative for gallstones but showed slight diffuse pancreatic edema. Magnetic resonance cholangiopancreatography (MRCP) showed dilation of the common hepatic duct to 10mm that then tapered to 2.5mm at the distal common bile duct. The pancreatic duct was unremarkable. Endoscopic retrograde cholangiopancreatography (ERCP) demonstrated a thread-like stricture of the common bile duct and a 10-French, 5cm Amsterdam biliary stent (Cook Medical Inc., Bloomington, IL) was placed. Bile duct brushings or biopsies were not taken. The patient’s pain and liver function tests improved and he was referred to our institution for further management.
A second ERCP was performed two months after the first because of the concern for cholangiocarcinoma. He was found to have a high-grade, 2.5cm-long, distal common bile duct stricture with proximal and distal shelving, suspicious for cholangiocarcinoma. Repeat biliary stenting was
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performed, and biliary brushings and intraductal biopsies, which were obtained during this procedure, were negative for dysplasia.
The outside CT scan was reviewed and demonstrated a bulbous appearing pancreas with a subtle peripancreatic halo (see Figure 1). No pancreatic mass was noted. The outside MRCP was again noted to demonstrate a high-grade stricture of the intrapancreatic portion of the common bile duct (see Figure 2). There was irregular beaded narrowing of the pancreatic duct in the body and tail of the pancreas. The diagnosis of cholangiocarcinoma was suspected but could not be proven. An endoscopic ultrasound (EUS) with fine-needle aspiration was therefore performed. A 26x18mm inhomogenous, hypoechoic pancreatic head mass with a 7x9mm adjacent lymph node and a 22x22mm well-defined hypoechoic mass in the pancreatic body were noted. Fine-needle aspirations were obtained from all three sites, but the cytology was non-diagnostic.
The patient was referred for a pancreaticoduodenectomy because the diagnosis of cholangiocarcinoma and, possibly, pancreatic adenocarcinoma was suspected, although not proven. The patient declined surgery and on subsequent examination he was found to have enlarged submandibular salivary glands bilaterally. Serum immunoglobulin G4 (IgG4) levels were within normal limits, 42.1mg/dl (RR 2.4–121). A positron emission tomography (PET) scan demonstrated multiple areas of hypermetabolism, including the uncinate process and the body and tail of the pancreas, the biliary tree, gallbladder wall, aorta, submandibular glands, ethmoid
© TOUCH BRIEFINGS 2011
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