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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 Inflammation: Introduction01:12

Chronic Inflammation: Introduction

Chronic inflammation is a prolonged, dysregulated immune response that persists for weeks to years when the inciting stimulus is difficult to eradicate or when self‑antigens drive ongoing reactivity. Morphologically, it is defined by mononuclear cell infiltration, progressive tissue destruction, and concurrent attempts at healing via angiogenesis and fibrosis. Compared with acute inflammation, edema is less prominent while cellular infiltration predominates; triggers include persistent...
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 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...
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 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...

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Related Experiment Video

Updated: Jun 23, 2026

An Adoptive Transfer Model of Rheumatoid Arthritis in Mice
07:37

An Adoptive Transfer Model of Rheumatoid Arthritis in Mice

Published on: June 6, 2025

[Autoinflammatory syndromes].

P Lamprecht1, W L Gross

  • 1Poliklinik für Rheumatologie, Vaskulitiszentrum UKSH & Klinikum Bad Bramstedt, Universität zu Lübeck, Ratzeburger Allee 160, 23538 Lübeck.

Der Internist
|May 13, 2009
PubMed
Summary
This summary is machine-generated.

Autoinflammatory syndromes, or hereditary periodic fever syndromes (HPF), stem from genetic mutations affecting pattern-recognition receptors (PRR) and cytokine balance, causing innate immune system overactivation. These syndromes manifest as recurrent fevers and inflammation, and PRR pathways are implicated in other chronic inflammatory conditions.

Related Experiment Videos

Last Updated: Jun 23, 2026

An Adoptive Transfer Model of Rheumatoid Arthritis in Mice
07:37

An Adoptive Transfer Model of Rheumatoid Arthritis in Mice

Published on: June 6, 2025

Area of Science:

  • Immunology
  • Genetics
  • Rheumatology

Context:

  • Autoinflammatory syndromes are a group of rare genetic disorders.
  • Hereditary periodic fever syndromes (HPF) are a subset of autoinflammatory syndromes.
  • These conditions involve dysregulation of the innate immune system.

Purpose:

  • To define and categorize autoinflammatory syndromes, focusing on hereditary periodic fever syndromes (HPF).
  • To elucidate the genetic basis, particularly mutations in pattern-recognition receptors (PRR), and cytokine imbalances.
  • To highlight the clinical manifestations and explore the role of PRR in broader inflammatory and autoimmune diseases.

Summary:

  • Autoinflammatory syndromes, strictly defined as hereditary periodic fever syndromes (HPF), arise from mutations in pattern-recognition receptors (PRR) and altered cytokine balance.
  • Specific HPF examples include cryopyrinopathies, familial Mediterranean fever, TRAPS, HIDS, PAPA syndrome, NALP12-HPF, and Blau syndrome.
  • These syndromes feature spontaneous innate immune cell activation without external triggers, typically presenting with recurrent fevers, inflammation of serosal/synovial interfaces, and skin lesions, and lack autoantibodies.

Impact:

  • Provides a clear definition and classification of autoinflammatory syndromes and HPF.
  • Establishes the central role of PRR mutations and cytokine dysregulation in the pathogenesis of HPF.
  • Suggests a potential link between PRR-mediated mechanisms and other chronic inflammatory and autoimmune diseases, opening avenues for research.