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Zierke et al. Rare Dis Orphan Drugs J 2023;2:10 Rare Disease and
DOI: 10.20517/rdodj.2022.17
Orphan Drugs Journal

Review Open Access

Pathogenesis of autoimmune and hereditary
pancreatitis with a focus on neutrophil granulocytes

and neutrophil serine proteases

1,2
1
1
Lukas Zierke , Marcel Gischke , Quang Trung Tran , Ali A. Aghdassi 1
1
Department of Medicine A, University Medicine Greifswald, Greifswald 17475, Germany.
2
Department of internal medicine, Hue University, Hue city 530000, Vietnam.
Correspondence to: Prof. Ali A. Aghdassi, Department of Medicine A, University Medicine Greifswald, Fleischmannstraße 8,
Greifswald 17475, Germany. E-mail: Ali.Aghdassi@med.uni-greifswald.de
How to cite this article: Zierke L, Gischke M, Tran QT, Aghdassi AA. Pathogenesis of autoimmune and hereditary pancreatitis
with a focus on neutrophil granulocytes and neutrophil serine proteases. Rare Dis Orphan Drugs J 2023;2:10.
https://dx.doi.org/10.20517/rdodj.2022.17
Received: 10 Oct 2022 First Decision: 13 Mar 2023 Revised: 20 Apr 2023 Accepted: 24 Apr 2023 Published: 28 Apr 2023

Academic Editors: Daniel Scherman, Brice Korkmaz Copy Editor: Ying Han Production Editor: Ying Han

Abstract
Hereditary and autoimmune pancreatitis are two rare forms of inflammatory pancreatic disorders. Both share
similarities with acute, acute recurrent, and chronic pancreatitis. Regarding their pathogenesis, the premature
activation of the digestive protease trypsinogen and the infiltration of inflammatory cells such as
polymorphonuclear leukocytes and macrophages into the pancreas are highly relevant and can reciprocally amplify
inflammation. Neutrophil serine proteases are the main components of neutrophil granulocytes and have different
pro-inflammatory effects in many diseases. However, their role in pancreatitis is still limited. This section focuses
on known findings regarding the role of this group of enzymes in hereditary and autoimmune pancreatitis.

Keywords: Hereditary pancreatitis, autoimmune pancreatitis, neutrophil serine proteases, cathepsin C

INTRODUCTION
Acute pancreatitis is defined as an acute inflammatory condition of the pancreas and is characterized by
self-digestion of the pancreatic tissue by its own digestive enzymes. It is one of the most frequent
gastrointestinal diseases leading to hospitalization in Europe and the United States, with an estimated

© The Author(s) 2023. Open Access This article is licensed under a Creative Commons Attribution 4.0
International License (https://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, sharing,
adaptation, distribution and reproduction in any medium or format, for any purpose, even commercially, as
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