Volume 14, Issue 3 (11-2024)
Clin Exc 2024, 14(3): 68-78 |
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Assari R, Ziaee V. Vasculitis in autoinflammatory disorders. Clin Exc 2024; 14 (3) :68-78
URL: http://ce.mazums.ac.ir/article-1-881-en.html
URL: http://ce.mazums.ac.ir/article-1-881-en.html
Pediatric Rheumatology, Pediatric Rheumatology Research Group, Rheumatology Research Center, Tehran University of Medical Sciences, Tehran, Iran.
Abstract: (250 Views)
Autoinflammatory diseases are a category of inflammatory conditions characterized by a disruption in the regulation of the myeloid lineage. Unlike autoimmune diseases, which involve dysregulation of the lymphoid lineage and are associated with antibody production, these conditions typically do not involve antibodies. Vasculitides and vasculopathies are observed in this category of inflammatory diseases, manifesting through various mechanisms. These manifestations may be due to the direct effects of the cytotoxicity of IL-1β, type 1 interferon, or the formation of inflammatory immune complexes on endothelial cells. Autoinflammatory diseases include conditions such as inflammasomopathies, DADA2 (deficiency of adenosine deaminase 2), and interferonopathies. Additionally, Behçet's disease and, in children, genetic disorders that present similar symptoms to Behçet's may also exhibit signs of vasculitis and vasculopathy. In treating these diseases, targeting the pathological pathways can provide better assistance to patients, and in some cases, a multi-drug approach may be utilized to control the condition. For genetic deficiencies and monogenic patients, bone marrow transplantation may ultimately benefit these individuals.
Type of Study: Review |
Subject:
روماتولوژی اطفال
Received: 2024/11/6 | Accepted: 2025/01/20 | Published: 2025/01/20
Received: 2024/11/6 | Accepted: 2025/01/20 | Published: 2025/01/20
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