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Related Concept Videos

Autoimmune Disorders01:29

Autoimmune Disorders

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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...
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Inflammatory Response I: Vascular and Cellular01:30

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The inflammatory response is the body's defense against infection, injury, or irritation from bacteria, trauma, toxins, or heat. Inflammation helps locate and destroy pathogens and remove damaged tissue elements to heal the body. During this initial phase, fluid, blood products, and nutrients migrate to the injured area, resulting in redness, heat, swelling, ache, and loss of function. Moreover, signs of systemic inflammation include fever, increased WBC count, malaise, anorexia, nausea,...
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Inflammatory Bowel Disease II: Crohn's Disease01:30

Inflammatory Bowel Disease II: Crohn's Disease

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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...
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Inflammatory Bowel Disease I: Ulcerative Colitis01:27

Inflammatory Bowel Disease I: Ulcerative Colitis

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Inflammatory bowel disease, or IBD, encompasses a group of disorders characterized by chronic inflammation or ulceration of the gastrointestinal tract.
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Chronic Bowel Disorders: Introduction01:17

Chronic Bowel Disorders: Introduction

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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.
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Rheumatic Heart Disease I: Introduction01:23

Rheumatic Heart Disease I: Introduction

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Rheumatic heart disease or RHD is a chronic condition that results from rheumatic fever, causing permanent damage to the heart valves.Etiology and Risk FactorsIt primarily arises from rheumatic fever, an inflammatory disease that can develop after untreated or inadequately treated group A streptococcal (GAS) pharyngitis. Streptococcus spreads through direct contact with oral or respiratory secretions. While the bacteria are the causative agents, factors like malnutrition, overcrowding, poor...
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Related Experiment Video

Updated: Sep 10, 2025

An Immunohistopathologic Study to Profile the Folate Receptor Beta Macrophage and Vascular Immune Microenvironment in Giant Cell Arteritis
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Vasculitis in autoinflammatory diseases.

Emilie Bohbot1, Eldad Ben-Chetrit

  • 1Rheumatology Unit, Division of Medicine, Hadassah-Hebrew University Medical Center, Jerusalem, Israel.

Current Opinion in Rheumatology
|August 26, 2025
PubMed
Summary
This summary is machine-generated.

Autoinflammatory diseases (AIDs) are linked to vasculitis, which is often missed. Recognizing these distinct patterns aids early diagnosis and tailored treatments for better outcomes.

Keywords:
autoinflammatory diseasesdeficiency of adenosine deaminase 2familial Mediterranean fevervacuoles, E1 enzyme, X-linked, autoinflammatory, somatic syndromevasculitis

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Area of Science:

  • Immunology
  • Rheumatology
  • Genetics

Background:

  • Autoinflammatory diseases (AIDs) involve innate immune system dysregulation.
  • Vasculitis in AIDs is frequently underrecognized and misdiagnosed.
  • Recent data since 2016 highlight newly identified syndromes and insights.

Purpose of the Study:

  • To explore the relationship between autoinflammatory diseases and vasculitis.
  • To focus on recently identified syndromes and updated data.
  • To review vasculitic manifestations in various AIDs.

Main Methods:

  • Literature review of recent publications (since 2016).
  • Analysis of clinical, genetic, and histologic features of AIDs with vasculitis.
  • Discussion of underlying inflammatory pathways and genetic mutations.

Main Results:

  • Vasculitis occurs in diverse AIDs, including familial Mediterranean fever, DADA2, VEXAS, and CAPS.
  • Vascular involvement ranges from cutaneous findings to systemic vasculitis.
  • Mechanisms involve IL-1β, NF-κB overactivation, and novel genetic mutations.

Conclusions:

  • Early recognition of vasculitis in AIDs is crucial for diagnosis, especially in atypical cases.
  • Genetic testing is recommended for suspected cases.
  • Understanding distinct patterns guides management with biologics or stem cell transplantation for improved outcomes.