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Related Concept Videos

Autoimmune Disorders01:29

Autoimmune Disorders

Autoimmune diseases are a group of disorders in which the body's immune system mistakenly attacks its own cells, tissues, and organs. This results from an overactive immune response against substances and tissues normally present in the body. Let's delve into the concept and mechanism of autoimmune diseases from an immune system point of view, explore different causes and examples of such diseases, and discuss potential solutions.
Concept and Mechanism of Autoimmune Diseases
The immune system...
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...
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 transmural...
Inflammatory Bowel Disease I: Introduction01:26

Inflammatory Bowel Disease I: Introduction

Inflammatory bowel disease is a group of chronic disorders marked by recurrent inflammation of the gastrointestinal tract due to an abnormal immune response against gut microflora. This leads to tissue damage. The two main forms are Crohn’s disease and ulcerative colitis.Crohn’s DiseaseCrohn’s disease is a relapsing inflammatory disorder that can affect any part of the GI tract, from the mouth to the anus. It involves all layers of the bowel wall (transmural) and shows “skip lesions” in which...
Inflammatory Bowel Disease II: Ulcerative Colitis01:20

Inflammatory Bowel Disease II: Ulcerative Colitis

Ulcerative colitis is a chronic inflammatory disorder of the colon characterized by continuous mucosal inflammation that typically begins in the rectum and extends proximally in a uniform pattern. Its pathogenesis involves a complex interplay of genetic predisposition, immune dysregulation, and environmental influences. These factors converge to impair the colon’s epithelial defenses and promote an exaggerated inflammatory response against luminal contents.Breakdown of the Mucosal BarrierA...
Inflammatory Bowel Disease III: Crohn's Disease01:25

Inflammatory Bowel Disease III: Crohn's Disease

Crohn’s disease is a chronic, relapsing form of inflammatory bowel disease characterized by segmental, transmural inflammation that can affect any part of the gastrointestinal tract. Its pathogenesis arises from a combination of genetic susceptibility, environmental exposures, epithelial barrier dysfunction, and immune dysregulation. Together, these factors lead to an exaggerated immune response against components of the gut microbiome.Genetic and Environmental InfluencesMultiple genetic...

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Related Experiment Video

Updated: May 12, 2026

Induction and Clinical Scoring of Chronic-Relapsing Experimental Autoimmune Encephalomyelitis
26:48

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Autoinflammatory Diseases: A Review.

Jason An1, Ashish Marwaha2, Ronald M Laxer3

  • 1J. An, MD, MSc, Division of Rheumatology, Hospital for Sick Children, University of Toronto, Toronto, Ontario; jason.an@sickkids.ca.

The Journal of Rheumatology
|June 15, 2024
PubMed
Summary
This summary is machine-generated.

Autoinflammatory diseases (AIDs) involve recurrent sterile inflammation. This review covers their complex pathophysiology, classification, and management, focusing on adult rheumatologists and mimicking rheumatic diseases.

Keywords:
autoinflammationautoinflammatory diseasesgeneticsinnate immunityperiodic feversrecurrent fevers

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Area of Science:

  • Immunology
  • Genetics
  • Rheumatology

Background:

  • Autoinflammatory diseases (AIDs) are a diverse group of disorders featuring recurrent sterile inflammation.
  • The discovery of new AIDs has accelerated since 1997, with 485 inborn errors of immunity documented by 2022, many exhibiting autoinflammatory features.

Purpose of the Study:

  • To review the current understanding of AID pathophysiology and propose a novel classification system.
  • To emphasize AIDs relevant to adult rheumatologists, including those mimicking systemic lupus erythematosus and inflammatory arthritis.
  • To provide an approach for clinical assessment, diagnosis, and management of AIDs.

Main Methods:

  • Literature review focusing on autoinflammatory diseases.
  • Analysis of genetic and environmental factors in AID pathophysiology.
  • Synthesis of information for classification and clinical management strategies.

Main Results:

  • AIDs exhibit complex pathophysiology, involving rare gene mutations or polygenic inheritance triggered by environmental factors.
  • AIDs present across all age groups, posing diagnostic challenges due to rarity and heterogeneity.
  • Many AIDs can mimic common rheumatic diseases, necessitating careful evaluation.

Conclusions:

  • A proposed classification system aims to organize the growing field of AIDs.
  • Understanding AID pathophysiology is crucial for accurate diagnosis and effective management.
  • Rheumatologists play a key role in managing AIDs, requiring awareness of their diverse presentations.