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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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The immune...
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Hypersensitivity, also known as a hypersensitivity reaction or allergic reaction, is a condition where the body's immune system reacts abnormally to a foreign substance. Such substances, that cause hypersensitivity are referred to as an allergen, could be something typically harmless to most people, like pollen or certain foods.
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Skin is the first line of defense and encounters a variety of microbes. Some pathogenic strains are often the cause of a broad range of infections of the skin and other body systems. These conditions can affect people of all ages and may have different causes, including genetic factors, infections, autoimmune reactions, environmental factors, and lifestyle choices.
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Allergic reactions related to drugs are hypersensitivity responses driven by the immune system and bear no connection to the drug's therapeutic action. While drugs in isolation do not trigger an immune response, they can interact with endogenous proteins to form antigens. These antigens stimulate lymphocytes to produce antibodies. IgE-type antibodies attach themselves to mast cells. Upon subsequent exposure to the same stimulus, the antigen-antibody interaction is initiated, unleashing...
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Basophil Activation Test for Allergy Diagnosis
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Autoimmune chronic spontaneous urticaria.

Pavel Kolkhir1, Melba Muñoz2, Riccardo Asero3

  • 1Institute of Allergology, Charité - Universitätsmedizin Berlin, corporate member of Freie Universität Berlin, and Humboldt-Universität zu Berlin, Berlin, Germany; Fraunhofer Institute for Translational Medicine and Pharmacology ITMP, Allergology and Immunology, Berlin, Germany; I.M. Sechenov First Moscow State Medical University (Sechenov University), Division of Immune-Mediated Skin Diseases, Moscow, Russia.

The Journal of Allergy and Clinical Immunology
|June 6, 2022
PubMed
Summary
This summary is machine-generated.

Chronic spontaneous urticaria (CSU) involves autoimmune mechanisms. Understanding Type I and Type IIb CSU subtypes aids in developing targeted therapies for this mast cell-driven disease.

Keywords:
BATBHRAChronic spontaneous urticariaIgEIgG-anti-TPOautoallergyautoimmunitymarkerstreatmenttype Itype IIb

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Area of Science:

  • Immunology
  • Dermatology
  • Mast Cell Biology

Background:

  • Chronic spontaneous urticaria (CSU) is a severe mast cell-mediated condition causing wheals and angioedema.
  • Two primary autoimmune pathways drive CSU: Type I (autoallergic) involving IgE autoantibodies and Type IIb involving activating IgG autoantibodies.
  • A subset of CSU patients exhibits characteristics of both autoimmune subtypes.

Purpose of the Study:

  • To delineate the distinct autoimmune mechanisms in Chronic Spontaneous Urticaria (CSU).
  • To characterize the clinical and immunological features of Type IIb autoimmune CSU.
  • To highlight emerging targeted therapies and future research directions for CSU.

Main Methods:

  • Review and synthesis of current literature on CSU pathogenesis.
  • Analysis of diagnostic criteria for Type IIb autoimmune CSU, including triple positivity tests.
  • Examination of clinical characteristics associated with Type IIb CSU, such as disease severity and comorbidities.

Main Results:

  • Type IIb autoimmune CSU, present in less than 10% of patients, shows higher disease severity and is linked to other autoimmune conditions.
  • Patients with Type IIb CSU often present with low IgE, high IgG-anti-TPO, basopenia, eosinopenia, and poor response to antihistamines and omalizumab.
  • Type IIb CSU demonstrates a favorable response to cyclosporine.

Conclusions:

  • Differentiating between Type I and Type IIb autoimmune CSU is crucial for understanding disease heterogeneity.
  • Type IIb CSU requires distinct therapeutic strategies, with cyclosporine showing promise.
  • Further research is needed to refine diagnostic markers and explore novel treatments like BTK inhibitors and anti-IL-4Rα therapies.