Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2020 Oct;70(10):699-711.
doi: 10.1111/pin.12994. Epub 2020 Aug 6.

Guidance for diagnosing autoimmune pancreatitis with biopsy tissues

Affiliations

Guidance for diagnosing autoimmune pancreatitis with biopsy tissues

Kenji Notohara et al. Pathol Int. 2020 Oct.

Abstract

The biopsy-based diagnosis of autoimmune pancreatitis (AIP) is difficult but is becoming imperative for pathologists due to the increased amount of endoscopic ultrasound-guided biopsy tissue. To cope with this challenge, we propose guidance for the biopsy diagnosis of type 1 AIP. This guidance is for pathologists and comprises three main parts. The first part includes basic issues on tissue acquisition, staining, and final diagnosis, and is intended for gastroenterologists as well. The second part is a practical guide for diagnosing type 1 AIP based on the AIP clinical diagnostic criteria 2018. Inconsistent histological findings, tips for evaluating IgG4 immunostaining and key histological features including the ductal lesion and others are explained. Storiform fibrosis and obliterative phlebitis are diagnostic hallmarks but are sometimes equivocal. Storiform fibrosis is defined as spindle-shaped cells, inflammatory cells and fine collagen fibers forming a flowing arrangement. Obliterative phlebitis is defined as fibrous venous obliteration with inflammatory cells. Examples of each are provided. The third part describes the differentiation of AIP from pancreatic ductal adenocarcinoma (PDAC), focusing on histological features of acinar-ductal metaplasia in AIP, which is an important mimicker of PDAC. This guidance will help standardize pathology reports of pancreatic biopsies for diagnosing type 1 AIP.

Keywords: IgG4-related disease; autoimmune pancreatitis; biopsy; endoscopic ultrasound-guided fine needle aspiration; immunohistochemistry; pancreatic carcinoma; pathology.

PubMed Disclaimer

References

REFERENCES

    1. Shimosegawa T, Chari ST, Frulloni L et al. International consensus diagnostic criteria for autoimmune pancreatitis: Guidelines of the International Association of Pancreatology. Pancreas 2011; 40: 352-58.
    1. The Japan Pancreas Society. The Research Program on Intractable Diseases from the Ministry of Labour and Welfare of Japan. Japanese Clinical Diagnostic Criteria for Autoimmune Pancreatitis, 2018 (Proposal); Revision of Japanese Clinical Diagnostic Criteria for Autoimmune Pancreatitis, 2011. J Jpn Panc Soc 2018; 33: 902-13. in Japanese.
    1. Kawa S, Kamisawa T, Notohara K et al. Japanese Clinical Diagnostic Criteria for Autoimmune Pancreatitis, 2018: Revision of Japanese Clinical Diagnostic Criteria for Autoimmune Pancreatitis, 2011. Pancreas 2020; 49: e13-e14.
    1. Kawaguchi K, Koike M, Tsuruta K, Okamoto A, Tabata I, Fujita N. Lymphoplasmacytic sclerosing pancreatitis with cholangitis: A variant of primary sclerosing cholangitis extensively involving pancreas. Hum Pathol 1991; 22: 387-95.
    1. Notohara K, Burgart LJ, Yadav D, Chari S, Smyrk TC. Idiopathic chronic pancreatitis with periductal lymphoplasmacytic infiltration: Clinicopathologic features of 35 cases. Am J Surg Pathol 2003; 27: 1119-27.

LinkOut - more resources