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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 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
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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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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Inflammatory Bowel Disease I: Ulcerative Colitis01:27

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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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T Cell Types and Functions01:24

T Cell Types and Functions

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When T cells with CD4 markers are activated, they give rise to two types of effector cells: helper T cells and regulatory T cells. Meanwhile, T cells with CD8 markers differentiate into effector cytotoxic T cells. The differentiation of CD4 T cells into helper T cell subsets, such as Th1, Th2, and Th17 cells, is dependent on the antigen type, antigen-presenting cell, and regulatory cytokines.
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Drugs for Treatment of Crohn's Disease in IBD Using Biologic Agents: Anti-TNF01:24

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

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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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Autoinflammatory Diseases in Childhood

Mehmet Yıldız1, Fatih Haşlak1, Amra Adrovic1

  • 1Department of Pediatric Rheumatology, İstanbul University-Cerrahpaşa Cerrahpaşa School of Medicine, İstanbul, Turkey

Balkan Medical Journal
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Autoinflammatory diseases involve recurrent fevers due to innate immunity defects, distinct from autoimmune conditions. Recent advances expand understanding of their genetics, pathogenesis, and treatment.

Keywords:
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Area of Science:

  • Immunology
  • Genetics
  • Rheumatology

Background:

  • Autoinflammatory diseases (AIDs) are characterized by recurrent fevers and impaired innate immunity, distinct from autoimmune diseases.
  • The understanding of AIDs has evolved significantly over the last two decades, driven by genetic and pathogenetic discoveries.
  • Familial Mediterranean fever and tumor necrosis factor receptor-associated periodic syndrome were early defined AIDs.

Purpose of the Study:

  • To review the clinical features, classification, and management of common autoinflammatory diseases.
  • To discuss the expanding spectrum of AIDs in light of recent molecular and genetic advancements.
  • To provide an overview of treatment options and long-term prognosis for periodic fever, aphthous stomatitis, pharyngitis, adenitis syndrome (PFAPA) and other AIDs.

Main Methods:

  • Literature review of current scientific publications.
  • Analysis of recent developments in molecular sciences and genetics related to AIDs.
  • Synthesis of information on clinical manifestations, diagnostic criteria, and therapeutic strategies.

Main Results:

  • AIDs encompass a broad range of conditions with diverse clinical presentations, primarily affecting innate immunity.
  • Genetic and pathogenetic insights have refined the classification and understanding of AIDs.
  • The spectrum of AIDs is rapidly expanding, necessitating updated diagnostic and treatment approaches.

Conclusions:

  • Autoinflammatory diseases represent a complex group of disorders with significant heterogeneity.
  • Continued research in genetics and molecular biology is crucial for advancing the diagnosis and treatment of AIDs.
  • A comprehensive understanding of AIDs is essential for effective patient management and improving long-term outcomes.