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Related Concept Videos

Chronic Pancreatitis II: Pathophysiology01:21

Chronic Pancreatitis II: Pathophysiology

Chronic pancreatitis is a progressive and irreversible inflammation of the pancreas, most often caused by long-term alcohol abuse, but it can also be related to ductal obstruction, smoking, or genetic factors.Chronic pancreatitis occurs when the pancreas is repeatedly exposed to harmful agents like alcohol, smoking, ductal obstruction, or genetic predisposition. These factors lead to the release of toxic metabolites and inflammatory cytokines, sustaining chronic inflammation in the pancreatic...
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Chronic Pancreatitis I: Introduction

Chronic pancreatitis is a long-standing, relapsing inflammation of the pancreas, characterized by irreversible damage to the gland. It results in progressive destruction of the pancreatic parenchyma, fibrosis, and eventual loss of both exocrine and endocrine function. The disease may evolve gradually after multiple episodes of acute pancreatitis or develop independently.EtiologyChronic pancreatitis can arise from a variety of causes:Alcohol use is the leading cause, accounting for 70–80% of...
Chronic Pancreatitis I: Introduction01:24

Chronic Pancreatitis I: Introduction

The pancreas, an elongated and flat gland situated behind the stomach, serves a vital function in digesting food and managing blood sugar levels.
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Acute pancreatitis arises suddenly and lasts for a brief duration, while chronic pancreatitis is a long-term affliction...
Cirrhosis II: Pathophysiology01:24

Cirrhosis II: Pathophysiology

Cirrhosis is a progressive chronic liver injury caused by prolonged inflammation, excessive fibrotic remodeling, and impaired regeneration. Over time, repeated hepatic insults disrupt the liver’s architecture and function, leading to reduced blood flow, impaired bile drainage, and diminished metabolic capacity.Pathophysiology of cirrhosisCirrhosis arises from three main responses to chronic liver damage: inflammation, immune activation, and hepatocyte death. These processes lead to structural...
Cholecystitis01:20

Cholecystitis

Cholecystitis is inflammation of the gallbladder, most commonly caused by obstruction of the cystic duct. This blockage prevents bile from draining, leading to gallbladder distension, inflammation, and potentially serious complications. This condition may present acutely or chronically and can happen with or without gallstones.EtiologyAbout 95% of cholecystitis cases are calculous, caused by gallstones blocking the cystic duct, leading to bile accumulation and inflammation of the gallbladder...
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Related Experiment Video

Updated: May 29, 2026

Bile Duct Ligation in Mice: Induction of Inflammatory Liver Injury and Fibrosis by Obstructive Cholestasis
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Published on: February 10, 2015

Recurrent liver failure caused by IgG4 associated cholangitis.

Jacob N Clendenon1, Jaime Aranda-Michel, Murli Krishna

  • 1Department of Transplantation, Mayo Clinic Florida, Jacksonville, Florida 32224, USA.

Annals of Hepatology
|September 14, 2011
PubMed
Summary

Immunoglobulin G4 associated cholangitis (IAC) mimics primary sclerosing cholangitis (PSC) but progresses faster. Early diagnosis and steroid treatment are crucial for managing this autoimmune liver disease and preventing liver failure.

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Area of Science:

  • Gastroenterology and Hepatology
  • Autoimmune Diseases
  • Transplant Surgery

Background:

  • Immunoglobulin G4 associated cholangitis (IAC) is an autoimmune condition often presenting similarly to primary sclerosing cholangitis (PSC).
  • IAC typically exhibits a more rapid symptom onset and higher incidence of obstructive jaundice compared to PSC.
  • Steroid therapy is a hallmark treatment for IAC, leading to disease remission.

Observation:

  • A 59-year-old female with undiagnosed IAC developed liver failure after a pancreaticoduodenectomy for suspected cancer, initially presumed to be PSC.
  • The patient underwent liver transplantation, but experienced allograft failure within five years due to progressive bile duct injury, with recurrent PSC suspected.
  • Diagnosis of IAC was confirmed via liver biopsy showing IgG4 positive cells, despite initial radiology and histology suggesting PSC recurrence.

Findings:

  • The patient underwent successful liver retransplantation and is currently on triple immunosuppressive therapy.
  • Recurrent PSC with atypical features, including a history of pancreatitis, warrants testing for IAC.
  • IAC is highly responsive to steroid therapy, suggesting its importance in management.

Implications:

  • This case highlights the importance of considering IAC in patients with suspected or recurrent PSC, especially those with atypical presentations or a history of pancreatitis.
  • Prompt diagnosis and initiation of steroid therapy for IAC can prevent disease progression and potentially avoid the need for retransplantation.
  • Further research into differentiating IAC from PSC and optimizing treatment protocols is warranted.