Behcet’s Disease: An In-Depth Review about Pathogenesis, Gastrointestinal Manifestations, and Management

Abstract

Background: Behcet’s Disease (BD) is a complex inflammatory vascular disorder that follows a relapsing-remitting course with diverse clinical manifestations. The prevalence of the disease varies throughout the globe and targets different age groups. There are many variations of BD, however, intestinal BD is not only more common but has many signs and symptoms. Summary: BD is a relapsing-remitting inflammatory vascular disorder with multiple system involvement, affecting vessels of all types and sizes that targets young adults. The etiology of BD is unknown but many factors including genetic mechanisms, vascular changes, hypercoagulability and dysregulation of immune function are believed to be responsible. BD usually presents with signs and symptoms of ulcerative disease of the small intestine; endoscopy being consistent with the clinical manifestations. The mainstay of treatment depends upon the severity of the disease. Corticosteroids are recommended for severe forms of the disease and aminosalicylic acids are used in maintaining remission in mild to moderate forms of the disease. Key messages: In this review, we have tried to summarize in the present review the clinical manifestations, differential diagnoses and management of intestinal BD. Hopefully, this review will enable health policymakers to ponder over establishing clear endpoints for treatment, surveillance investigations and creating robust algorithms.

Hemorragia de vías digestivas bajas en un paciente con enfermedad de Behçet: reporte de un caso

“…La histopatología de la EB a nivel gastrointestinal tiene diversas características, se presenta con hallazgos de infiltrados mononuclear y de mastocitos perivasculares, vasculitis neutrofílica y cambios inflamatorios agudos y crónicos en la submucosa intestinal (15) . Aunque en el reporte histopatológico de las biopsias tomadas durante la endoscopia nes gastrointestinales (14) .…”

“…En relación con lo anterior, la afectación gastrointestinal de la EB varía dependiendo de la localización del tracto gastrointestinal. El compromiso esofágico es poco frecuente y se manifiesta por úlceras, estenosis y perforación; a nivel del estómago y del duodeno se presentan úlceras duodenales; en el yeyuno, íleon y colon son predominantes las complicaciones relacionadas con perforación, hemorragias digestivas, fístulas, estenosis y abscesos, y el íleon terminal y la región ileocecal son las porciones intestinales más afectadas (15)(16)(17) . Adicionalmente, se han documentado linfangiectasias intestinales con enteropatía perdedora de proteínas (18) .…”

Diverse selection pressures shaping the genetic architecture of behçet disease susceptibility

“…Recognition of infectious organisms' proteins or human proteins due to molecular mimicry (similarity between pathogen antigens and human peptides) by the innate immune system is suggested to be a trigger in the pathogenesis of BD (Mattioli et al, 2021;Nguyen et al, 2021). Only in East Asian populations, we found signals of recent selective sweep in genes involved in intracellular pattern recognition (NOD2), intracellular processing of (foreign) peptides (DTL, UBASH3B, GALNT10, LYST), immune regulation (HLA-G), regulation of cellular growth and migration (CTNNA2, NAV2, SEMA6D, RALGAPA2), and differentiation of B cells (EBF2).…”

Manifestations of the gastrointestinal tract in systemic rheumatic diseases: A narrative review

“…При ББ, как и при БК, имеется ассоциация с наличием антител к Saccharomyces cerevisae (ASCA, anti-Saccharomyces cerevisiae antibodies). Диагностика поражения тонкой кишки при ББ может вызывать серьезные трудности и требовать применения высокотехнологичных диагностических методов, таких как видеокапсульная эндоскопия (ВКЭ) [2,29,30]. Любопытно, что частота вовлечения тонкой и толстой кишки при ББ зависит от популяции и географической зоны.…”

“…В отличие от БК, эти изменения не носят сегментарного или сплошного характера, а при гистологическом исследовании не определяются характерные для БК гранулемы. Помимо изменений ЖКТ, при ББ может развиваться патология других органов пищеварительной системы -печени, желчевыводящих путей и поджелудочной железы [2,29,30].…”