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Experimental Therapeutic Solutions for Behcet’s Disease
Behcet’s disease (BD) is a chronic systemic vasculitis with inflammation attacks that involve multiple organs. In addition to numerous mucocutaneous symptoms, notably recurrent oral and genital ulcers, ocular, articular, vascular, gastrointestinal, cardiac, and neurological system involvement can be...
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Formato: | Online Artículo Texto |
Lenguaje: | English |
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Dove
2021
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Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7886245/ https://www.ncbi.nlm.nih.gov/pubmed/33603502 http://dx.doi.org/10.2147/JEP.S265645 |
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author | Bozca, Burçin Cansu Alpsoy, Erkan |
author_facet | Bozca, Burçin Cansu Alpsoy, Erkan |
author_sort | Bozca, Burçin Cansu |
collection | PubMed |
description | Behcet’s disease (BD) is a chronic systemic vasculitis with inflammation attacks that involve multiple organs. In addition to numerous mucocutaneous symptoms, notably recurrent oral and genital ulcers, ocular, articular, vascular, gastrointestinal, cardiac, and neurological system involvement can be observed. Mucocutaneous lesions are the primary symptom of the disease in most patients, and they usually occur before major organ involvement and other symptoms of the disease. Recognizing the disease’s mucocutaneous lesions is very important to diagnose at an early stage, control with appropriate treatment and close follow-up, and prevent major organ involvement. Genome-wide association studies (GWAS) in recent years have confirmed that HLA-B*51 is the most significant genetic predisposing factor. The majority of gene polymorphisms have been detected in molecules that respond to microorganisms and genes encoding cytokines and adhesion molecules. The infectious agent S. sanguinis -commonly found in the oral mucosa of patients with BD- or the differences in the salivary or intestinal microbiome composition can trigger innate immune-mediated inflammation sustained by acquired or adaptive immune responses. In antigen-presenting cells (APCs), epistatic interactions between HLA-B*51 and endoplasmic reticulum aminopeptidase 1 (ERAP1) variants lead to the disruption of T-cell homeostasis, especially the activation of Type1 T-helper and Th17 pathway and suppression of regulatory T-cells. Recent developments to clarify the disease’s etiopathogenesis provided us with a better understanding of the mechanism of action of the relatively old drugs while opening a way for many new treatment methods. Apremilast has become an important option in the treatment of mucocutaneous symptoms with its high efficacy and safety. The disease increases the mortality rate, especially in young male patients. New treatments, especially anti-TNF-α agents, have provided significant progress and decreased the mortality rates with their rapid effect and high efficacy in patients with severe organ involvement and resistance to traditional immunosuppressive and immunomodulatory therapies. The use of IL-1, IL-6, IL-17, IL-12/IL-23 antagonists in different organ involvement has gradually increased, and the quality of life has significantly improved in many patients. |
format | Online Article Text |
id | pubmed-7886245 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2021 |
publisher | Dove |
record_format | MEDLINE/PubMed |
spelling | pubmed-78862452021-02-17 Experimental Therapeutic Solutions for Behcet’s Disease Bozca, Burçin Cansu Alpsoy, Erkan J Exp Pharmacol Review Behcet’s disease (BD) is a chronic systemic vasculitis with inflammation attacks that involve multiple organs. In addition to numerous mucocutaneous symptoms, notably recurrent oral and genital ulcers, ocular, articular, vascular, gastrointestinal, cardiac, and neurological system involvement can be observed. Mucocutaneous lesions are the primary symptom of the disease in most patients, and they usually occur before major organ involvement and other symptoms of the disease. Recognizing the disease’s mucocutaneous lesions is very important to diagnose at an early stage, control with appropriate treatment and close follow-up, and prevent major organ involvement. Genome-wide association studies (GWAS) in recent years have confirmed that HLA-B*51 is the most significant genetic predisposing factor. The majority of gene polymorphisms have been detected in molecules that respond to microorganisms and genes encoding cytokines and adhesion molecules. The infectious agent S. sanguinis -commonly found in the oral mucosa of patients with BD- or the differences in the salivary or intestinal microbiome composition can trigger innate immune-mediated inflammation sustained by acquired or adaptive immune responses. In antigen-presenting cells (APCs), epistatic interactions between HLA-B*51 and endoplasmic reticulum aminopeptidase 1 (ERAP1) variants lead to the disruption of T-cell homeostasis, especially the activation of Type1 T-helper and Th17 pathway and suppression of regulatory T-cells. Recent developments to clarify the disease’s etiopathogenesis provided us with a better understanding of the mechanism of action of the relatively old drugs while opening a way for many new treatment methods. Apremilast has become an important option in the treatment of mucocutaneous symptoms with its high efficacy and safety. The disease increases the mortality rate, especially in young male patients. New treatments, especially anti-TNF-α agents, have provided significant progress and decreased the mortality rates with their rapid effect and high efficacy in patients with severe organ involvement and resistance to traditional immunosuppressive and immunomodulatory therapies. The use of IL-1, IL-6, IL-17, IL-12/IL-23 antagonists in different organ involvement has gradually increased, and the quality of life has significantly improved in many patients. Dove 2021-02-12 /pmc/articles/PMC7886245/ /pubmed/33603502 http://dx.doi.org/10.2147/JEP.S265645 Text en © 2021 Bozca and Alpsoy. http://creativecommons.org/licenses/by-nc/3.0/ This work is published and licensed by Dove Medical Press Limited. The full terms of this license are available at https://www.dovepress.com/terms.php and incorporate the Creative Commons Attribution – Non Commercial (unported, v3.0) License (http://creativecommons.org/licenses/by-nc/3.0/). By accessing the work you hereby accept the Terms. Non-commercial uses of the work are permitted without any further permission from Dove Medical Press Limited, provided the work is properly attributed. For permission for commercial use of this work, please see paragraphs 4.2 and 5 of our Terms (https://www.dovepress.com/terms.php). |
spellingShingle | Review Bozca, Burçin Cansu Alpsoy, Erkan Experimental Therapeutic Solutions for Behcet’s Disease |
title | Experimental Therapeutic Solutions for Behcet’s Disease |
title_full | Experimental Therapeutic Solutions for Behcet’s Disease |
title_fullStr | Experimental Therapeutic Solutions for Behcet’s Disease |
title_full_unstemmed | Experimental Therapeutic Solutions for Behcet’s Disease |
title_short | Experimental Therapeutic Solutions for Behcet’s Disease |
title_sort | experimental therapeutic solutions for behcet’s disease |
topic | Review |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7886245/ https://www.ncbi.nlm.nih.gov/pubmed/33603502 http://dx.doi.org/10.2147/JEP.S265645 |
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