Practice of Pancreatic Enzyme Replacement Therapy in Patients With Exocrine Insufficiency; A North European Survey

Abstract

Paraffin-embedded specimens were stained for IgG4 and reviewed by two GI pathologists. AIP was confirmed based on the presence of lymphoplasmocytic infiltrate, obliterative phlebitis, with or without positive IgG4 staining (≥10 IgG4+ plasma cells/hpf). Patients who were treated with corticosteroids were excluded. The medical records were reviewed for clinical and follow-up information. The national death index was queried for mortality. Results: We identified 17 patients with AIP who have been evaluated at our institute from 1997 to 2010. All patients had their AIP confirmed by histologic examination of pancreatic tissue obtained by Whipple procedure (n=5), distal pancreatectomy (n=6), or biopsy (n= 6). 4 cases were from 2006 to 2010 and the remaining cases from 1997 to 2005. Of note, only 6 cases had an initial histologic diagnosis of AIP and all of them were from 2005 or later. The median age at presentation was 55 years (range: 18-78) and 58.8% were men. None of these patients received corticosteroid treatment. The patients who had surgical resection have been followed up for a median 39 months [range 1-165 months; mean 48.1 (±49.2)]. In this group, 1 patient developed CP, 3 developed biliary involvement, 1 acute recurrent pancreatitis, and 3 developed AIP-related autoimmune diseases [ulcerative colitis (1); hypothyroidism (2)] during the follow-up period. One patient died at 110 months after the initial diagnosis. The patients who had biopsy have been followed up for a median 42 months [range (1-122); mean 54.3 (±51.0)], one had persistent biliary involvement and one developed exocrine deficiency 7 years after the diagnosis. None of these patients developed pancreatic cancer during follow-up. The Kaplan-Meier survival curve is shown in figure 1. Conclusions: Our results highlight the persistence and progressive nature of AIP during its natural course and suggest AIP is not associated with high risk of pancreatic adenocarcinoma. Clinical implication: histologic material of “usual” chronic pancreatitis should be reviewed retrospectively by GI pathologists if the disease is persistent and progressive for an optimal clinical management of patients with AIP.