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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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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.
Th1 cells stimulate dendritic cells to express necessary co-stimulatory molecules on their surfaces for...
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Inflammatory Bowel Disease I: Ulcerative Colitis01:27

Inflammatory Bowel Disease I: Ulcerative Colitis

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Introduction
Inflammatory bowel disease, or IBD, encompasses a group of disorders characterized by chronic inflammation or ulceration of the gastrointestinal tract.
Risk Factors
The exact cause of IBD remains unclear, although it is believed to be due to a mix of genetic, environmental, microbial, and immune factors. Genetic factors are significant in determining susceptibility to IBD, with family history being a critical risk factor. Individuals with a first-degree relative who has IBD are at...
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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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Inflammation01:38

Inflammation

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Related Experiment Video

Updated: Jul 9, 2025

Author Spotlight: Exploring the Role of Inflammation in the Co-occurrence of Primary Sjogren's Syndrome and Lung Adenocarcinoma
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Author Spotlight: Exploring the Role of Inflammation in the Co-occurrence of Primary Sjogren's Syndrome and Lung Adenocarcinoma

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[Lung involvement in autoinflammatory diseases].

M-L Frémond1, L Berteloot2, A Hadchouel3

  • 1Unité d'immuno-hématologie et rhumatologie pédiatriques, hôpital Necker-Enfants-Malades, AP-HP, centre université de Paris-Cité, 149, rue de Sèvres, 75015 Paris, France; Institut imagine, laboratoire de neurogénétique et neuroinflammation, université de Paris-Cité, 24, boulevard du Montparnasse, 75015 Paris, France.

Revue Des Maladies Respiratoires
|December 1, 2023
PubMed
Summary
This summary is machine-generated.

Genetic autoinflammatory diseases increasingly affect the lungs, leading to conditions like pulmonary fibrosis. Molecular diagnostics and targeted therapies, such as JAK inhibitors and methionine supplementation, offer new hope for these rare lung diseases.

Keywords:
Alveolar proteinosisAutoinflammatory diseasesCOPAHémorragie intra-alvéolaireInhibiteurs de JAKInterferonInterféronInterstitial lung diseaseIntra-alveolar hemorrhageJAK inhibitorsMARSMaladies auto-inflammatoiresMethionineMéthioninePneumopathie interstitielleProtéinose alvéolaireSTINGSeritisSérite

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Area of Science:

  • Genetics
  • Immunology
  • Pulmonology

Context:

  • Genetic autoinflammatory diseases are a growing field.
  • Historically, lung involvement manifested as inflammatory seritis.
  • Recent discoveries highlight pulmonary manifestations in type I interferonopathies like SAVI and COPA syndrome.

Purpose:

  • To review the spectrum of lung involvement in genetic autoinflammatory diseases.
  • To discuss the diagnostic and therapeutic implications of a molecular approach.
  • To highlight emerging therapeutic strategies for pulmonary manifestations.

Summary:

  • Pulmonary involvement is a significant feature in several genetic autoinflammatory diseases, including SAVI, COPA syndrome, and MARS mutation-associated alveolar proteinosis.
  • These conditions often lead to progressive pulmonary fibrosis, contributing to high morbidity and mortality.
  • VEXAS syndrome also shows frequent pulmonary involvement in adults.
  • A molecular approach aids in diagnosis and identifies targeted treatments, such as JAK inhibitors and methionine supplementation.

Impact:

  • Advances in understanding genetic autoinflammatory diseases are revealing the lung as a key affected organ.
  • Molecular diagnostics are crucial for identifying specific disease subtypes and guiding therapy.
  • Targeted treatments show promise, though preventing fibrosis remains a challenge.
  • Ongoing research is expected to uncover more lung pathologies and novel therapeutic avenues.