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Adamantiades–Behçet disease (ABD) is a rare, multisystem inflammatory disease of unknown aetiology, classified as a systemic vasculitis and as a neutrophilic dermatosis involving all types and sizes of blood vessels. A genetically determined background with environmental triggering factor(s) are probably involved. A chronic, relapsing, progressive course of oral aphthous ulcers, genital ulcers, skin lesions (papulopustules, erythema nodosum) and iridocyclitis/posterior uveitis is characteristic. ABD is occasionally accompanied by arthritis and vascular, neurological and gastrointestinal manifestations. ABD has a worldwide occurrence with varying prevalence, being endemic in eastern and central Asian and the eastern Mediterranean countries. Characteristic histopathological features of ABD are vasculitis and thrombosis. ABD is not considered contagious. There is no specific mode of Mendelian transmission in ABD. The disease has potentially poor prognosis (especially in males with systemic presenting signs). HLA‐B51 and ERAP‐1 seem to be associated with a more severe prognosis. Immunological mechanisms are considered to play a major role in the pathogenesis of ABD. The disease has currently been classified among the autoinflammatory disorders. The endothelium seems to be the primary target. Diagnosis of ABD is based on clinical signs. The Revised International Criteria for Behçet's Disease provides the most accurate diagnosis. Ophthalmic and neurological sequelae are leading causes of morbidity, followed by severe vascular and gastrointestinal manifestations. Their effects on morbidity may be cumulative. The clinical course of ABD is variable. A multidisciplinary approach in the management of ABD patients is mandatory. The choice of treatment depends on the site and severity of the clinical manifestations of the disease.
Adamantiades–Behçet disease (ABD) is a rare, multisystem inflammatory disease of unknown aetiology, classified as a systemic vasculitis and as a neutrophilic dermatosis involving all types and sizes of blood vessels. A genetically determined background with environmental triggering factor(s) are probably involved. A chronic, relapsing, progressive course of oral aphthous ulcers, genital ulcers, skin lesions (papulopustules, erythema nodosum) and iridocyclitis/posterior uveitis is characteristic. ABD is occasionally accompanied by arthritis and vascular, neurological and gastrointestinal manifestations. ABD has a worldwide occurrence with varying prevalence, being endemic in eastern and central Asian and the eastern Mediterranean countries. Characteristic histopathological features of ABD are vasculitis and thrombosis. ABD is not considered contagious. There is no specific mode of Mendelian transmission in ABD. The disease has potentially poor prognosis (especially in males with systemic presenting signs). HLA‐B51 and ERAP‐1 seem to be associated with a more severe prognosis. Immunological mechanisms are considered to play a major role in the pathogenesis of ABD. The disease has currently been classified among the autoinflammatory disorders. The endothelium seems to be the primary target. Diagnosis of ABD is based on clinical signs. The Revised International Criteria for Behçet's Disease provides the most accurate diagnosis. Ophthalmic and neurological sequelae are leading causes of morbidity, followed by severe vascular and gastrointestinal manifestations. Their effects on morbidity may be cumulative. The clinical course of ABD is variable. A multidisciplinary approach in the management of ABD patients is mandatory. The choice of treatment depends on the site and severity of the clinical manifestations of the disease.
Background This study aimed to investigate the expression profile of immune response-related proteins of Behcet’s disease (BD) patients and identify potential biomarkers for this disease. Methods Plasma was collected from BD patients and healthy controls (HC). Immune response-related proteins were measured using the Olink Immune Response Panel. Differentially expressed proteins (DEPs) were used to construct prediction models via five machine learning algorithms: naive Bayes, support vector machine, extreme gradient boosting, random forest, and neural network. The prediction performance of the five models was assessed using the area under the curve (AUC) value, recall (sensitivity), specificity, precision, accuracy, F1 score, and residual distribution. Subtype analysis of BD was performed using the consensus clustering method. Results Proteomics results showed 43 DEPs between BD patients and HC (P < 0.05). These DEPs were mainly involved in the Toll-like receptor 9 and NF-κB signaling pathways. Five models were constructed using DEPs [interleukin 10 (IL10), Fc receptor like 3 (FCRL3), Mannan-binding lectin serine peptidase 1 (MASP1), NF2, moesin-ezrin-radixin like (MERLIN) tumor suppressor (NF2), FAM3 metabolism regulating signaling molecule B (FAM3B), and O-6-methylguanine-DNA methyltransferase (MGMT)]. Among these models, the neural network model showed the best performance (AUC = 0.856, recall: 0.692, specificity: 0.857, precision: 0.900, accuracy: 0.750, F1 score: 0.783). BD patients were divided into two subtypes according to the consensus clustering method: one with high disease activity in association with higher expression of tripartite motif-containing 5 (TRIM5), SH2 domain-containing 1A (SH2D1A), phosphoinositide-3-kinase adaptor protein 1 (PIK3AP1), hematopoietic cell-specific Lyn substrate 1 (HCLS1), and DNA fragmentation factor subunit alpha (DFFA) and the other with low disease activity in association with higher expression of C–C motif chemokine ligand 11 (CCL11). Conclusions Our study not only revealed a distinctive immune response-related protein profile for BD but also showed that IL10, FCRL3, MASP1, NF2, FAM3B, and MGMT could serve as potential immune biomarkers for this disease. Additionally, a novel molecular disease classification model was constructed to identify subsets of BD.
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