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The Challenge of Autoimmune Pancreatitis

A Portrayal From the Pediatric Perspective

Pagliari, Danilo, MD*; Cianci, Rossella, MD, PhD; Rigante, Donato, MD, PhD‡§

doi: 10.1097/MPA.0000000000001307

Autoimmune pancreatitis (AIP) is a rare disorder characterized by prompt clinical response to corticosteroids. Lost tolerance to a variety of pancreatic antigens and subsequent development of autoantibodies are presumably involved in the initiation of AIP. Even pediatric patients have been reported with features of AIP, and awareness of this disorder is increasing among different clinicians. The terms lymphoplasmacytic sclerosing pancreatitis and idiopathic duct-centric pancreatitis refer to the different histologic patterns of AIP, named type 1 and type 2, respectively. A combination of serologic, radiologic, and histologic investigations is needed to assess diagnosis of AIP and rule out neoplastic disorders. In addition, type 1 AIP can be distinguished by raised levels of serum immunoglobulin G4 and should be considered as part of systemic immunoglobulin G4–related disease. Conversely, type 2 AIP is frequently reported in younger patients and has less clear immune-mediated pathogenetic mechanisms. The natural history of pediatric AIP is obscure, and the diagnostic usefulness of different autoimmune abnormalities found in adults with AIP is limited for children. Tips to manage pediatric patients with AIP have been recently drafted through a set of recommendation statements. This review describes the current data about AIP and the pathogenic contribution of specific autoantibodies expressly in the pediatric population.

From the Institutes of *Internal Medicine and Gastroenterology and Pancreatic Unit,

Internal Medicine, and

Pediatrics, Fondazione Policlinico Universitario A. Gemelli, IRCCS; and

§Università Cattolica Sacro Cuore, Rome, Italy.

Received for publication September 17, 2018; accepted March 20, 2019.

Address correspondence to: Danilo Pagliari, MD, Fondazione Policlinico Universitario A. Gemelli, IRCCS, Largo A. Gemelli, 8, 00168 Rome, Italy (e-mail:

The authors declare no conflict of interest or funding.

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