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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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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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Inflammatory Bowel Disease II: Crohn's Disease01:30

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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
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Inflammatory Bowel Disease I: Ulcerative Colitis01:27

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Drugs for Treatment of Crohn's Disease in IBD Using Biologic Agents: Anti-TNF01:24

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Tumor Necrosis Factor (TNF), a proinflammatory cytokine, contributes significantly to the inflammation seen in Crohn's disease. It exists as soluble TNF and membrane-bound TNF, with actions mediated through TNF receptors (TNFR). TNFR activation leads to the release of proinflammatory cytokines, T-cell activation, collagen production, and leukocyte migration, all contributing to inflammation in Crohn's disease. Anti-TNF monoclonal antibodies, namely infliximab (Remicade), adalimumab...
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Rheumatic Heart Disease I: Introduction01:23

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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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Chronic Salmonella Infection Induced Intestinal Fibrosis
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Inherited Autoinflammatory Syndromes.

Julia E Rood1, Edward M Behrens1

  • 1Division of Rheumatology, Children's Hospital of Philadelphia, Perelman School of Medicine at The University of Pennsylvania, Philadelphia, Pennsylvania 19104, USA;

Annual Review of Pathology
|October 26, 2021
PubMed
Summary
This summary is machine-generated.

Autoinflammation involves immune system overactivation. This review categorizes inherited autoinflammatory diseases into five groups, aiding in understanding mechanisms and guiding future diagnostics and therapies.

Keywords:
geneticsimmunologyinflammation

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Area of Science:

  • Immunology
  • Genetics
  • Rheumatology

Background:

  • Autoinflammation comprises diverse diseases driven by non-antigen-specific immune system activation.
  • Advances in genetic diagnostics have identified numerous monogenic causes of autoinflammation.
  • Despite varied clinical presentations, autoinflammatory diseases can be grouped by underlying mechanisms.

Purpose of the Study:

  • To categorize archetypal inherited autoinflammatory diseases.
  • To provide a framework for understanding disease mechanisms.
  • To guide future research in diagnostics and therapeutics.

Main Methods:

  • Review of archetypal inherited autoinflammatory diseases.
  • Thematic grouping of diseases into five major categories.
  • Analysis of disease mechanisms and therapeutic targets.

Main Results:

  • Categorization of autoinflammatory diseases into inflammasomopathy, interferonopathy, unfolded protein/cellular stress response, relopathy, and uncategorized groups.
  • Identification of common themes in disease mechanisms across categories.
  • Highlighting areas for future research and therapeutic development.

Conclusions:

  • A five-category framework aids in understanding inherited autoinflammation.
  • This classification can inform the development of novel diagnostics.
  • The framework supports the exploration of targeted therapeutic strategies for autoinflammatory conditions.