Medical Department II, University Hospital, LMU, Munich, Germany; Department of Medicine A, University Medicine Greifswald, Greifswald, Germany.
Department of Medicine A, University Medicine Greifswald, Greifswald, Germany.
Gastroenterology. 2019 May;156(7):1951-1968.e1. doi: 10.1053/j.gastro.2018.11.081. Epub 2019 Jan 18.
Since the discovery of the first trypsinogen mutation in families with hereditary pancreatitis, pancreatic genetics has made rapid progress. The identification of mutations in genes involved in the digestive protease-antiprotease pathway has lent additional support to the notion that pancreatitis is a disease of autodigestion. Clinical and experimental observations have provided compelling evidence that premature intrapancreatic activation of digestive proteases is critical in pancreatitis onset. However, disease course and severity are mostly governed by inflammatory cells that drive local and systemic immune responses. In this article, we review the genetics, cell biology, and immunology of pancreatitis with a focus on protease activation pathways and other early events.
自遗传性胰腺炎家族中首次发现胰蛋白酶原突变以来，胰腺遗传学取得了快速进展。消化酶-抗蛋白酶途径相关基因的突变鉴定进一步支持了胰腺炎是一种自身消化性疾病的观点。临床和实验观察提供了令人信服的证据，表明消化蛋白酶在胰脏内过早激活在胰腺炎发病中起关键作用。然而，疾病进程和严重程度主要由炎症细胞驱动，炎症细胞引发局部和全身免疫反应。本文重点讨论蛋白酶激活途径和其他早期事件，综述了胰腺炎的遗传学、细胞生物学和免疫学。
