A peculiar case of autoimmune pancreatitis and sclerosing cholangitis

Abstract

A 65-year-old man presented with recurrent abdominal pain and slight weight loss. Computed tomography (CT) and endoscopic ultrasound (EUS) revealed a hypodense, partially cystic mass located in the pancreatic body and tail that raised the suspicion of pancreatic cancer (• " Fig. 1). No distant metastases were detected and the patient underwent surgical resection of the mass and splenectomy, which was complicated by a bleeding splenic artery aneurysm. Surprisingly, only massive inflammation of the pancreas was found on histological examination of the resected specimen. The patient re-presented 1 year later with jaundice for the first time. EUS demonstrated a pseudocyst within the head of the pancreas. Endoscopic retrograde cholangiopancreatography (ERCP) revealed an isolated stenosis of the distal common bile duct (• " Fig. 2 a). This was successfully treated with regularly exchanged endoprotheses, leading to a complete normalization of laboratory parameters. The patient returned 2 years later with fever and jaundice. This time ERCP showed a purulent cholangitis with a stricture of the biliary bifurcation as well as several stenotic intrahepatic bile ducts (• " Fig. 2 b). Brush cytology raised a suspicion of cholangiocarcinoma. Serum IgG4 levels, antinuclear antibody (ANA), antineutrophil cytoplasmic antibody (ANCA), anticarbonic anhydrase II and antilactoferrin antibodies were all within the normal range; however, there was peripheral eosinophilia, which can be associated with autoimmune pancreatitis (AIP) [1]. Histological re-examination of the surgical specimen revealed an impressive lymphoplasmacytic infiltration of the resected pancreas with IgG4-positive cells (• " Fig. 3 a, b). This led finally to the correct diagnosis of AIP type 1 with metachronous autoimmune cholangitis. The patient was treated according to the recently published diagnostic algorithms [2 -4] with long-term, slowly tapered prednisone, with resulting improvement in his clinical symptoms, biliary strictures (• " Fig. 2 c), blood eosinophilia, and laboratory parameters. In summary, distinguishing AIP from pancreatic cancer remains a pitfall for clinicians; in some cases, surgical resection remains the treatment of choice because IgG4 levels can be elevated in 10 % of pancreatic cancers [5].

Clinical presentation and organ-based outcomes of Multifocal fibrosclerosis: A systematic review

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