Autoimmune pancreatitis: Current perspectives

Over the last two decades, our knowledge and understanding regarding the pathogenesis and biology of autoimmune pancreatitis (AIP) have improved tremendously. Type 1 AIP or IgG4-related pancreatitis (IgG4-RP) is now believed to be the prototype of the multisystemic IgG4-related disease. In view of c...

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Autores principales:	Surbhi Goyal, Puja Sakhuja
Formato:	article
Lenguaje:	EN
Publicado:	Wolters Kluwer Medknow Publications 2021
Materias:	autoimmune pancreatitis histology idiopathic duct-centric pancreatitis igg4-related disease igg4-related sclerosing cholangitis igg4-related inflammatory pseudotumor lymphoplasmacytic pancreatitis Pathology RB1-214 Microbiology QR1-502
Acceso en línea:	https://doaj.org/article/0db6a472d2cd479d8f45192181f4b8ca
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spelling	oai:doaj.org-article:0db6a472d2cd479d8f45192181f4b8ca2021-12-02T19:27:27ZAutoimmune pancreatitis: Current perspectives0377-492910.4103/ijpm.ijpm_59_21https://doaj.org/article/0db6a472d2cd479d8f45192181f4b8ca2021-01-01T00:00:00Zhttp://www.ijpmonline.org/article.asp?issn=0377-4929;year=2021;volume=64;issue=5;spage=149;epage=159;aulast=Goyalhttps://doaj.org/toc/0377-4929Over the last two decades, our knowledge and understanding regarding the pathogenesis and biology of autoimmune pancreatitis (AIP) have improved tremendously. Type 1 AIP or IgG4-related pancreatitis (IgG4-RP) is now believed to be the prototype of the multisystemic IgG4-related disease. In view of clinical features like obstructive jaundice and mass-forming lesions in the pancreas in elderly men, type 1 AIP often mimics pancreatic cancer. IgG4-related sclerosing cholangitis concomitantly involving the extrahepatic and intrahepatic biliary tree is the most common extrapancreatic involvement seen in up to 80% of these patients, which needs to distinguish from cholangiocarcinoma. Histology is characterised by lymphoplasmacytic inflammation, abundant IgG4 positive plasma cell infiltration, storiform fibrosis and obliterative phlebitis. Apart from histology, high serum IgG4 levels, pancreatic parenchymal and duct imaging findings and other organ involvement aid in diagnosis especially in cases where definitive histology is not evident. Also, these parameters lay the foundation of various diagnostic criteria proposed over last few years. On the contrary, histology alone is the mainstay for establishing diagnosis of idiopathic duct-centric pancreatitis (IDCP) as it lacks any specific serological marker or imaging. Since both types of AIP respond dramatically to corticosteroid treatment, a biopsy is crucial to establish the preoperative diagnosis and to exclude malignancy so as to avoid unnecessary surgery. This review discusses the morphologic spectrum, treatment and prognosis of IgG4-RP and IDCP with an emphasis on approach to diagnosis with relevant histologic features, differential diagnoses and the challenges faced during biopsy interpretation.Surbhi GoyalPuja SakhujaWolters Kluwer Medknow Publicationsarticleautoimmune pancreatitishistologyidiopathic duct-centric pancreatitisigg4-related diseaseigg4-related sclerosing cholangitisigg4-related inflammatory pseudotumorlymphoplasmacytic pancreatitisPathologyRB1-214MicrobiologyQR1-502ENIndian Journal of Pathology and Microbiology, Vol 64, Iss 5, Pp 149-159 (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	autoimmune pancreatitis histology idiopathic duct-centric pancreatitis igg4-related disease igg4-related sclerosing cholangitis igg4-related inflammatory pseudotumor lymphoplasmacytic pancreatitis Pathology RB1-214 Microbiology QR1-502
spellingShingle	autoimmune pancreatitis histology idiopathic duct-centric pancreatitis igg4-related disease igg4-related sclerosing cholangitis igg4-related inflammatory pseudotumor lymphoplasmacytic pancreatitis Pathology RB1-214 Microbiology QR1-502 Surbhi Goyal Puja Sakhuja Autoimmune pancreatitis: Current perspectives
description	Over the last two decades, our knowledge and understanding regarding the pathogenesis and biology of autoimmune pancreatitis (AIP) have improved tremendously. Type 1 AIP or IgG4-related pancreatitis (IgG4-RP) is now believed to be the prototype of the multisystemic IgG4-related disease. In view of clinical features like obstructive jaundice and mass-forming lesions in the pancreas in elderly men, type 1 AIP often mimics pancreatic cancer. IgG4-related sclerosing cholangitis concomitantly involving the extrahepatic and intrahepatic biliary tree is the most common extrapancreatic involvement seen in up to 80% of these patients, which needs to distinguish from cholangiocarcinoma. Histology is characterised by lymphoplasmacytic inflammation, abundant IgG4 positive plasma cell infiltration, storiform fibrosis and obliterative phlebitis. Apart from histology, high serum IgG4 levels, pancreatic parenchymal and duct imaging findings and other organ involvement aid in diagnosis especially in cases where definitive histology is not evident. Also, these parameters lay the foundation of various diagnostic criteria proposed over last few years. On the contrary, histology alone is the mainstay for establishing diagnosis of idiopathic duct-centric pancreatitis (IDCP) as it lacks any specific serological marker or imaging. Since both types of AIP respond dramatically to corticosteroid treatment, a biopsy is crucial to establish the preoperative diagnosis and to exclude malignancy so as to avoid unnecessary surgery. This review discusses the morphologic spectrum, treatment and prognosis of IgG4-RP and IDCP with an emphasis on approach to diagnosis with relevant histologic features, differential diagnoses and the challenges faced during biopsy interpretation.
format	article
author	Surbhi Goyal Puja Sakhuja
author_facet	Surbhi Goyal Puja Sakhuja
author_sort	Surbhi Goyal
title	Autoimmune pancreatitis: Current perspectives
title_short	Autoimmune pancreatitis: Current perspectives
title_full	Autoimmune pancreatitis: Current perspectives
title_fullStr	Autoimmune pancreatitis: Current perspectives
title_full_unstemmed	Autoimmune pancreatitis: Current perspectives
title_sort	autoimmune pancreatitis: current perspectives
publisher	Wolters Kluwer Medknow Publications
publishDate	2021
url	https://doaj.org/article/0db6a472d2cd479d8f45192181f4b8ca
work_keys_str_mv	AT surbhigoyal autoimmunepancreatitiscurrentperspectives AT pujasakhuja autoimmunepancreatitiscurrentperspectives
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Autoimmune pancreatitis: Current perspectives

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