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Review
. 2024 Feb 28;30(8):817-832.
doi: 10.3748/wjg.v30.i8.817.

Autoimmune pancreatitis: Cornerstones and future perspectives

Camilla Gallo  1 , Giulia Dispinzieri  1 , Nicola Zucchini  2 , Pietro Invernizzi  1 , Sara Massironi  3
Affiliations
Review

Autoimmune pancreatitis: Cornerstones and future perspectives

Camilla Gallo et al. World J Gastroenterol. .

Abstract

Autoimmune pancreatitis (AIP) is an autoimmune subtype of chronic pancreatitis resulting from the aberrant immune response against the pancreas, leading to inflammation and fibrosis. Although AIP is rare, its incidence is increasing and is often misdiagnosed as other pancreatic diseases. AIP is commonly classified into two types. Type 1 AIP (AIP-1) is typically associated with elevated serum immunoglobulin G4 (IgG4) levels and systemic manifestations, while type 2 AIP is typically a more localized form of the disease, and may coexist with other autoimmune disorders, especially inflammatory bowel diseases. Additionally, there is emerging recognition of a third type (type 3 AIP), which refers to immunotherapy-triggered AIP, although this classification is still gaining acceptance in medical literature. The clinical manifestations of AIP mainly include painless jaundice and weight loss. Elevated serum IgG4 levels are particularly characteristic of AIP-1. Diagnosis relies on a combination of clinical, laboratory, radiological, and histological findings, given the similarity of AIP symptoms to other pancreatic disorders. The mainstay of treatment for AIP is steroid therapy, which is effective in most cases. Severe cases might require additional imm-unosuppressive agents. This review aims to summarize the current knowledge of AIP, encompassing its epidemiology, etiology, clinical presentation, diagnosis, and treatment options. We also address the challenges and controversies in diagnosing and treating AIP, such as distinguishing it from pancreatic cancer and managing long-term treatment, highlighting the need for increased awareness and knowledge of this complex disease.

Keywords: Autoimmune pancreatitis; Autoimmunity; Immunoglobulin G4; Pancreatitis; Relapse; Steroids.

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Conflict of interest statement

Conflict-of-interest statement: Authors declare no conflict of interests for this article.

Figures

Figure 1
Figure 1
Ethiopathology of different types of autoimmune pancreatitis. Ethiopathological mechanisms of type 1 autoimmune pancreatitis, type 2 autoimmune pancreatitis, and type 3 autoimmune pancreatitis. AIP: Autoimmune pancreatitis; IFN: Interferon; IL: Interleukine; TLRs: Toll-like receptors; pDCs: Plasmacytoid dendritic cells; IgG4: Immunoglobulin G4; AIP-1: Type 1 autoimmune pancreatitis; AIP-2: Type 2 autoimmune pancreatitis; AIP-4: Type 3 autoimmune pancreatitis.
Figure 2
Figure 2
Radiological appearance of autoimmune pancreatitis-part 1. A: Unenhanced computed tomography scan appearance of a diffuse autoimmune pancreatitis (AIP): Sausage-like swelling of the parenchyma, with straight margins, and consequent loss of the typical lobular structure. A hypodense fibrotic capsule-like rim demarcates the swollen pancreas (blue arrow); B: Unenhanced T1 weighted magnetic resonance imaging (MRI) appearance of a diffuse AIP: Sausage-like swelling of the parenchyma, with straight margins, and rectangular shape of the tail (cut-tail sign) (orange arrow); C: Arterial phase of the contrast-enhanced T1 weighted MRI: Homogeneous reduced enhancement of the pancreatic parenchyma, with a more hypointense fibrotic capsule-like rim that demarcates the swollen pancreas (orange arrow).
Figure 3
Figure 3
Radiological appearance of autoimmune pancreatitis-part 2. A: Unenhanced T1 weighted magnetic resonance imaging (MRI) images of autoimmune pancreatitis (AIP): Diffuse hypointense pancreas, with an even more hypointense fibrotic capsule-rim; B: Unenhanced T2 weighted MRI images of diffuse AIP: The affected parenchyma shows a moderately higher intensity signal, with a persistently low-intensity fibrotic rim.
Figure 4
Figure 4
Endoscopic ultrasound appearance of autoimmune pancreatitis. Endoscopic ultrasound aspect of a mass-forming autoimmune pancreatitis: Solitary, irregular hypoechoic mass, located in the head of the pancreas, without upstream dilatation of the main pancreatic duct.
Figure 5
Figure 5
Histology of autoimmune pancreatitis. A: Histological samples of type 1 autoimmune pancreatitis. Hematoxylin eosin (HE) 4, Duct centric lymphoplasmacytic infiltrate; B: HE 10, storiform fibrosis with intense lymphoplasmacytic infiltrate and obliterative phlebitis (arrow); C: HE 4, lobule complete effacement by inflammatory cells and fibrosis; D: Immunoglobulin G4 (IgG4) IIC 20, moderate increase of IgG4+ plasma cells.
See this image and copyright information in PMC

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