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Mucocutaneous manifestations of Behçet’s disease: Pathogenesis and management from perspectives of vasculitis

Behçet’s disease (BD) is a systemic inflammatory disorder characterized by vasculitis affecting blood vessels of any caliber or type. It can present with a wide spectrum of vasculitic lesions, including erythema nodosum-like lesions and retinal vasculitis, and may also lead to larger vessel diseases...

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Autores principales: Kim, Doyoung, Nakamura, Koichiro, Kaneko, Fumio, Alpsoy, Erkan, Bang, Dongsik
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9755684/
https://www.ncbi.nlm.nih.gov/pubmed/36530905
http://dx.doi.org/10.3389/fmed.2022.987393
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author Kim, Doyoung
Nakamura, Koichiro
Kaneko, Fumio
Alpsoy, Erkan
Bang, Dongsik
author_facet Kim, Doyoung
Nakamura, Koichiro
Kaneko, Fumio
Alpsoy, Erkan
Bang, Dongsik
author_sort Kim, Doyoung
collection PubMed
description Behçet’s disease (BD) is a systemic inflammatory disorder characterized by vasculitis affecting blood vessels of any caliber or type. It can present with a wide spectrum of vasculitic lesions, including erythema nodosum-like lesions and retinal vasculitis, and may also lead to larger vessel diseases, such as aortic aneurysm and deep vein thrombosis. The full etiology of BD remains unclear, but it is considered a polygenetic disease with multiple genetic risk factors that promote immune dysregulation and thrombophilia. Inflammation can be triggered by environmental factors, such as bacteria or viruses, and the dysregulation of innate and adaptive immune cell subsets. Neutrophils and lymphocytes are the primary players involved in BD pathogenesis, with specific innate (i.e., neutrophil-derived reactive oxygen species and neutrophil extracellular traps) and adaptive (i.e., anti-endothelial cell antibodies) processes inducing endothelial cell activation and chemotaxis of inflammatory cells, leading to coagulation and vasculitis. These inflammation-induced vasculitic or vasculopathic features are observed in most mucocutaneous BD lesions, although vasculitis per se is often pathologically evident only during a brief period of the disease process. Due to the multifactorial nature of BD-associated inflammation, broad-spectrum anti-inflammatory medications, including glucocorticoids and immunosuppressive drugs, have been the mainstay for managing BD. In addition, inhibitors of interleukin (IL)-1, tumor necrosis factor (TNF)-α, and IL-17, which target innate and adaptive immune functions dysregulated in BD, have emerged as promising new therapeutics. In this review, we discuss the muco-cutaneous manifestations of BD by focusing on the underlying vasculitic components in their pathologies, as well as the current array of treatment options.
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spelling pubmed-97556842022-12-17 Mucocutaneous manifestations of Behçet’s disease: Pathogenesis and management from perspectives of vasculitis Kim, Doyoung Nakamura, Koichiro Kaneko, Fumio Alpsoy, Erkan Bang, Dongsik Front Med (Lausanne) Medicine Behçet’s disease (BD) is a systemic inflammatory disorder characterized by vasculitis affecting blood vessels of any caliber or type. It can present with a wide spectrum of vasculitic lesions, including erythema nodosum-like lesions and retinal vasculitis, and may also lead to larger vessel diseases, such as aortic aneurysm and deep vein thrombosis. The full etiology of BD remains unclear, but it is considered a polygenetic disease with multiple genetic risk factors that promote immune dysregulation and thrombophilia. Inflammation can be triggered by environmental factors, such as bacteria or viruses, and the dysregulation of innate and adaptive immune cell subsets. Neutrophils and lymphocytes are the primary players involved in BD pathogenesis, with specific innate (i.e., neutrophil-derived reactive oxygen species and neutrophil extracellular traps) and adaptive (i.e., anti-endothelial cell antibodies) processes inducing endothelial cell activation and chemotaxis of inflammatory cells, leading to coagulation and vasculitis. These inflammation-induced vasculitic or vasculopathic features are observed in most mucocutaneous BD lesions, although vasculitis per se is often pathologically evident only during a brief period of the disease process. Due to the multifactorial nature of BD-associated inflammation, broad-spectrum anti-inflammatory medications, including glucocorticoids and immunosuppressive drugs, have been the mainstay for managing BD. In addition, inhibitors of interleukin (IL)-1, tumor necrosis factor (TNF)-α, and IL-17, which target innate and adaptive immune functions dysregulated in BD, have emerged as promising new therapeutics. In this review, we discuss the muco-cutaneous manifestations of BD by focusing on the underlying vasculitic components in their pathologies, as well as the current array of treatment options. Frontiers Media S.A. 2022-12-02 /pmc/articles/PMC9755684/ /pubmed/36530905 http://dx.doi.org/10.3389/fmed.2022.987393 Text en Copyright © 2022 Kim, Nakamura, Kaneko, Alpsoy and Bang. https://creativecommons.org/licenses/by/4.0/This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
spellingShingle Medicine
Kim, Doyoung
Nakamura, Koichiro
Kaneko, Fumio
Alpsoy, Erkan
Bang, Dongsik
Mucocutaneous manifestations of Behçet’s disease: Pathogenesis and management from perspectives of vasculitis
title Mucocutaneous manifestations of Behçet’s disease: Pathogenesis and management from perspectives of vasculitis
title_full Mucocutaneous manifestations of Behçet’s disease: Pathogenesis and management from perspectives of vasculitis
title_fullStr Mucocutaneous manifestations of Behçet’s disease: Pathogenesis and management from perspectives of vasculitis
title_full_unstemmed Mucocutaneous manifestations of Behçet’s disease: Pathogenesis and management from perspectives of vasculitis
title_short Mucocutaneous manifestations of Behçet’s disease: Pathogenesis and management from perspectives of vasculitis
title_sort mucocutaneous manifestations of behçet’s disease: pathogenesis and management from perspectives of vasculitis
topic Medicine
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9755684/
https://www.ncbi.nlm.nih.gov/pubmed/36530905
http://dx.doi.org/10.3389/fmed.2022.987393
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