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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.
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Tuberculosis, often called TB, is a contagious illness primarily caused by Mycobacterium tuberculosis. It mainly affects the lung parenchyma but can also impact other body parts.
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Tuberculosis (TB) is a contagious infection primarily affecting the lung parenchyma but which can also affect other body parts. TB can be classified based on disease development, presentation, and the affected anatomical site.
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T Cell Types and Functions01:24

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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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Tuberculosis, or TB, is a bacterial infectious disease caused by Mycobacterium tuberculosis. While its primary impact is on the lungs, leading to pulmonary tuberculosis, it can also affect various other organs, a condition referred to as extrapulmonary tuberculosis.
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Autoimmune Tubulopathies.

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  • 1Centre de Recherche des Cordeliers, INSERM, Sorbonne Université, Université Paris Cité, Paris, France; CNRS ERL 8228 - Laboratoire de Physiologie Rénale et Tubulopathies, Paris, France; Assistance Publique-Hôpitaux de Paris, Hôpital Européen Georges Pompidou, Service de Physiologie, Paris, France; Centre de Référence des Maladies Rénales Héréditaires de l'Enfant et de l'Adulte (MARHEA), Paris, France; Centre de Référence des Maladies Rares du Calcium et du Phosphate, Paris, France; and Faculté de Médecine, Université Paris Cité, Paris, France.

Journal of the American Society of Nephrology : JASN
|January 9, 2025
PubMed
Summary
This summary is machine-generated.

Autoimmune tubulopathies, caused by autoantibodies targeting kidney tubules, may be underdiagnosed. This review classifies these rare diseases by the affected kidney segment, aiding diagnosis and patient care.

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Area of Science:

  • Nephrology
  • Immunology
  • Pathology

Background:

  • Renal tubules and collecting ducts express numerous proteins susceptible to autoantibody attack.
  • Autoimmune tubulopathies are hypothesized to be underdiagnosed despite the potential for autoimmunity.
  • Understanding these conditions is crucial for accurate diagnosis and patient management.

Purpose of the Study:

  • To review current knowledge on autoimmune tubulopathies.
  • To classify autoimmune tubulopathies based on the targeted renal segment.
  • To highlight the need for increased physician awareness and further research.

Main Methods:

  • Literature review of autoimmune tubulopathies.
  • Classification of diseases by affected renal tubule segment.
  • Summary of clinical presentations and associated autoantibodies.

Main Results:

  • Autoantibodies can target proximal tubules, causing Fanconi syndrome, proximal tubular acidosis, or tubulointerstitial nephritis and uveitis syndrome.
  • Targeting the thick ascending limb may lead to acquired Bartter syndrome or hypomagnesemia with hypercalciuria.
  • Distal tubule autoantibodies are linked to acquired Gitelman syndrome, while collecting duct autoantibodies can cause distal tubular acidosis or nephrogenic diabetes insipidus.

Conclusions:

  • Autoimmune tubulopathies present with diverse phenotypes depending on the targeted renal segment.
  • Characterization of autoantibodies and pathogenesis often remains incomplete.
  • Increased physician awareness and research are vital for improving diagnosis and care of these rare autoimmune kidney diseases.