Inflammatory bowel disease and primary sclerosing cholangitis: One disease or two?

Kim N van Munster1, Annika Bergquist2, Cyriel Y Ponsioen1

  • 1Department of Gastroenterology and Hepatology, Amsterdam UMC, University of Amsterdam, the Netherlands.

Journal of Hepatology
|November 8, 2023
PubMed

Insights

Primary sclerosing cholangitis (PSC) and inflammatory bowel disease (IBD) share a strong connection, yet their exact relationship and underlying causes remain unclear. This review explores the epidemiology, phenotype, and potential mechanisms linking these two complex conditions.

Area of Science:

  • Hepatology and Gastroenterology
  • Autoimmune and Inflammatory Diseases

Background:

  • Primary sclerosing cholangitis (PSC) remains a significant unmet need in liver disease management.
  • PSC frequently co-occurs with inflammatory bowel disease (IBD), particularly extensive colitis.
  • The pathophysiology connecting PSC and IBD is not fully understood.

Purpose of the Study:

  • To review the epidemiology and specific clinical phenotype of IBD in PSC patients.
  • To explore potential pathophysiological mechanisms linking PSC and IBD.
  • To discuss the implications for managing patients with both PSC and IBD.

Main Methods:

  • Comprehensive literature review of studies investigating the association between PSC and IBD.
  • Analysis of epidemiological data, clinical features, and proposed pathophysiological links.
  • Discussion of current understanding and future research directions.

Main Results:

  • A high prevalence of IBD is observed in PSC patients, often presenting as extensive colitis.
  • The exact nature of the PSC-IBD relationship is debated: a shared susceptibility or PSC as an IBD manifestation.
  • Understanding the connection is crucial for effective management strategies.

Conclusions:

  • The co-occurrence of PSC and IBD highlights a complex interplay between liver and gut inflammation.
  • Further research is needed to elucidate the shared etiological factors and guide therapeutic interventions.
  • Distinguishing whether PSC and IBD are one or two diseases is critical for patient care.

Related Concept Videos

Chronic Bowel Disorders: Introduction01:17

Chronic Bowel Disorders: Introduction

Chronic bowel diseases are a group of long-term conditions affecting the digestive tract, characterized by inflammation and damage to the gut lining. These conditions primarily include irritable bowel syndrome and inflammatory bowel disease.
Irritable Bowel Syndrome (IBS) is a common disorder affecting the gastrointestinal tract. The distinctive feature is recurrent abdominal pain associated with altered bowel movements, manifesting as constipation, diarrhea, or fluctuating between both. The...
458
Inflammatory Bowel Disease I: Ulcerative Colitis01:27

Inflammatory Bowel Disease I: Ulcerative Colitis

Introduction
Inflammatory bowel disease, or IBD, encompasses a group of disorders characterized by chronic inflammation or ulceration of the gastrointestinal tract.
Risk Factors
The exact cause of IBD remains unclear, although it is believed to be due to a mix of genetic, environmental, microbial, and immune factors. Genetic factors are significant in determining susceptibility to IBD, with family history being a critical risk factor. Individuals with a first-degree relative who has IBD are at...
192
Inflammatory Bowel Disease II: Crohn's Disease01:30

Inflammatory Bowel Disease II: Crohn's Disease

Introduction
Inflammatory bowel disease, commonly known as IBD, refers to a collection of disorders that lead to persistent inflammation of the gastrointestinal tract. The two types of IBD are ulcerative colitis, which impacts the colon, and Crohn's disease, which can involve any part of the gastrointestinal segment.
Crohn's disease
Crohn's disease is a chronic, systemic inflammatory bowel disease (IBD) that predominantly affects the gastrointestinal tract. It is marked by...
259
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.
Pancreatitis is the inflammation of the pancreas, which occurs when the immune system becomes active and causes swelling, pain, and disruptions in organ function. Pancreatitis can manifest as either an acute or chronic condition.
Acute pancreatitis arises suddenly and lasts for a brief duration, while chronic pancreatitis is a long-term affliction...
93
Inflammatory Bowel Disease III: Diagnostic Studies and Management I-Nutritional Therapy01:30

Inflammatory Bowel Disease III: Diagnostic Studies and Management I-Nutritional Therapy

Various diagnostic tests are employed in the diagnostic process for Inflammatory Bowel Disease (IBD), particularly to differentiate between Crohn's disease and ulcerative colitis.
Diagnostic studies
A colonoscopy is the definitive screening test, distinguishing ulcerative colitis from other colon diseases with similar symptoms. During a colonoscopy test, inflamed mucosa with exudate ulcerations can be observed, and biopsies are taken to determine the histologic characteristics of the...
338
Inflammatory Bowel Disease V: Surgical Management01:21

Inflammatory Bowel Disease V: Surgical Management

Surgical interventions for inflammatory bowel disease (IBD), which includes ulcerative colitis and Crohn's disease, are essential in managing symptoms and addressing complications. The selection of surgical procedures is contingent upon the specific conditions and complications that stem from these illnesses.
Here are some common surgical interventions for IBD:
149