Behçet’s disease is far more than recurrent mouth ulcers: this evidence map shows it as a whole-body inflammatory vasculitis that can threaten vision, veins, arteries, brain, gut, and quality of life. The full review explains which diagnostic signals, genetic mimics, and modern therapies — especially anti-TNF agents, apremilast, colchicine, and emerging JAK inhibitors — may change how Behçet’s disease is recognized and treated.
Human-verified editorial review
Verified by World ID proof-of-human. This editorial layer was submitted from a SAIMSARA account verified as a unique human.
Abstract: To synthesize current evidence regarding the pathophysiology, clinical manifestations, diagnostic biomarkers, and therapeutic strategies for Behçet's disease based on a structured extraction of multi-center cohorts, case-control studies, and clinical trials. The review utilises 3695 original studies with 103639919 total participants (topic deduplicated ΣN). The mapped evidence supports Behçet's disease as a polygenic autoinflammatory vasculitis in which HLA-B*51–ERAP1 interactions, neutrophil hyperactivation with NETosis, and monocyte–macrophage dysregulation converge to drive multisystem injury, with the International Criteria for Behçet's Disease showing higher diagnostic sensitivity/coverage (81.0%–88.1%) than the older ISG criteria (43.3%–59.1%) and Paediatric Behçet's Disease criteria (37.3%). Across topics, anti-TNF therapy emerged as a recurrent signal of benefit in refractory ocular, vascular, neurologic, and intestinal disease, with vascular remission reported in approximately 89% of cases and adalimumab plus corticosteroids superior to ciclosporin for severe uveitis. Apremilast and emerging JAK inhibitors (baricitinib, upadacitinib) were associated with improvement in mucocutaneous and refractory intestinal/vascular phenotypes, while colchicine retained a role for mucocutaneous and joint manifestations. Diagnostic signals including common femoral vein wall thickness, OCT angiography, deep-learning endoscopy, and proteomic/metabolomic panels indicate a shift toward multimodal, non-invasive phenotyping, although venous thrombosis (~14% of patients), arterial aneurysms, and neuro-Behçet's disease continue to drive morbidity and mortality, particularly in young males. The map also highlights underrecognized burdens of fatigue, depression, sexual dysfunction, and monogenic Behçet-like mimics that warrant integrated psychosocial care and targeted genetic screening in early-onset or refractory presentations. Given the heterogeneity of small retrospective cohorts and the absence of pooled effect estimation, future work should prioritize multi-center randomized trials of JAK inhibitors, longitudinal microbiome studies, and standardized anticoagulation–immunosuppression protocols to resolve the most persistent uncertainties identified across this evidence map.
Final search date and database lock: 2026-05-03 11:33:13 CEST
Plan: Pro (expanded craft tokens; source: PubMed)
Source: PubMed
Total Abstracts/Papers: 10195
Downloaded Abstracts/Papers: 10000
Included original and non-original Abstracts/Papers (all): 4326
Included original Abstracts/Papers (Vote counting by direction of effect): 3695
Reference Index (links used in paper): 457
Total participants (topic deduplicated ΣN): 103639919
Get access to the full paper
Unlock the full evidence map
The full evidence review, including the Introduction, Methods, Results, Discussion, Conclusion, figures, and complete reference index, opens after purchase or sign-in.
The Evidence Object JSON is a separate machine-readable evidence product: a concentrated synthesis of results, topic-level evidence, and discussion across original and non-original studies. It can be directly input into your LLM, agent, or RAG workflow.
[98] Case report: Successful remission with upadacitinib in a young patient with anti-TNF-refractory intestinal Behçet's disease. — https://doi.org/10.3389/fimmu.2024.1483993
[153] Characteristics of late-onset Behçet's disease and comparison with juvenile and adult-onset Behçet's disease. — https://doi.org/10.1111/ijd.17456
[216] Ocular Manifestations of Behçet's Disease: An Update on Diagnostic Challenges and Disease Management. — https://doi.org/10.3390/jcm10215174
[232] Possible Case of Elderly-onset Intestinal Behçet's Disease with Trisomy 8 Following COVID-19 Vaccination Exacerbated by COVID-19 Infection. — https://doi.org/10.2169/internalmedicine.4197-24
[259] Rapid Progression of Large B-cell Lymphoma in Behçet's Disease on Immunosuppressive Therapy: A Case Report with Literature Review. — https://doi.org/10.7759/cureus.28029
[298] International consensuses and guidelines on managing ocular Behçet's disease by the Academy of Asia-Pacific Professors of Ophthalmology (AAPPO), the Asia-Pacific Vitreo-Retina Society (APVRS), the Asia-Pacific Society of Ocular Inflammation and Infection (APSOII) and the Academia Retina Internationalis (ARI). — https://doi.org/10.1016/j.apjo.2025.100261
[352] Efficacy of anti-TNF alpha in severe and refractory major vessel involvement of Behcet's disease: A multicenter observational study of 18 patients. — https://doi.org/10.1016/j.clim.2018.08.004
[359] Allogeneic stem cell transplantation for trisomy 8-positive myelodysplastic syndrome or myelodysplastic/myeloproliferative disease with refractory Behçet's disease: Case report and the review of literature. — https://doi.org/10.1093/mrcr/rxac032
[391] Decreasing incidence and severity of Behçet’s disease: a changing trend in epidemiological spectrum possibly associated with oral health. — https://doi.org/10.3906/sag-2003-147
[439] Cluster analysis of paediatric Behçet's disease: Data from The Pediatric Rheumatology Academy-Research Group. — https://doi.org/10.1093/mr/roac044
[514] Allogeneic hematopoietic stem cell transplantation for juvenile myelomonocytic leukemia with intestinal Behçet's disease: A case report. — https://doi.org/10.1111/1756-185x.14882
[538] Large pulmonary artery pseudoaneurysm in a patient with Behçet's disease treated with an Amplatzer vascular plug. — https://doi.org/10.1136/bcr-2019-229516
[608] A case with Behçet's disease involving erosive Metacarpophalangeal joint arthritis: the value of ultrasonography in the diagnosis of an Erosion. — https://doi.org/10.1186/s12880-020-00461-8
[614] Bilateral Renal Artery Thrombosis in a Patient With Behçet's Disease Managed With Infliximab Infusions. — https://doi.org/10.7759/cureus.105037
[618] Successful Treatment of Behçet's Disease Associated with Acute Myeloid Leukemia with Myelodysplasia-related Changes Using Azacitidine and Tacrolimus before Allogeneic Hematopoietic Stem Cell Transplantation. — https://doi.org/10.2169/internalmedicine.56.7808
[677] Real-World Safety and Effectiveness of Infliximab in 255 Patients with Intestinal, Neurological, and Vascular Behçet's Disease: A Post-Marketing Surveillance. — https://doi.org/10.1007/s12325-024-02993-9
[730] Clinical and Demographic Features of Pediatric-Onset Behçet's Disease and Evaluation of Optical Coherence Tomography Findings. — https://doi.org/10.1080/09273948.2019.1611875
[732] Efficacy and tolerability of methotrexate therapy for refractory intestinal Behçet's disease: a single center experience. — https://doi.org/10.5217/ir.2018.16.2.315
[750] "Deficiency in ELF4, X-Linked": a Monogenic Disease Entity Resembling Behçet's Syndrome and Inflammatory Bowel Disease. — https://doi.org/10.1007/s10875-023-01610-8
[853] Age-related differences in the clinical phenotypes of Behçet's disease: The experience of two referral centres. — https://doi.org/10.1093/mr/road012
[939] The Frequency of Fibromyalgia and its Relationship With Disease Activity in Female Patients With Behçet's Disease: A Cross Sectional Study. — https://doi.org/10.46497/archrheumatol.2020.7729
[1008] Long-term maintenance of the mucosal healing induced by azacitidine therapy in a patient with intestinal Behçet's-like disease accompanied with myelodysplastic syndrome involving trisomy 8. — https://doi.org/10.1080/25785826.2019.1687251
[1041] Computed tomography enterography increases the ability of endoscopy to differentiate Crohn's disease from intestinal Behçet's disease. — https://doi.org/10.3389/fmed.2022.900458
[1087] The Relationship between Fluorescein Angiography Leakage after Infliximab Therapy and Relapse of Ocular Inflammatory Attacks in Ocular Behçet's Disease Patients. — https://doi.org/10.1080/09273948.2019.1641611
[1125] Aortic graft invasion into duodenum as an atypical pathergy phenomenon in vascular Behçet's disease. — https://doi.org/10.1093/mrcr/rxag004
[1128] Abnormal glucose and lipid metabolism promotes disrupted differentiation of T and B cell subsets in Behçet's disease. — https://doi.org/10.1093/immadv/ltaf010
[1130] Investigation of pregnancy associated plasma protein-A and neopterin levels in Behçet's patients. — https://doi.org/10.1111/dth.13443
[1149] Gastric Focal Neutrophil Infiltration and Wide Duodenal Gastric Foveolar Metaplasia Are Histologic Discriminative Markers for Crohn's Disease and Behçet's Disease. — https://doi.org/10.1159/000494922
[1232] Long-term clinical outcomes of intestinal Behçet's disease: A 30-year cohort study at a tertiary hospital in South Korea. — https://doi.org/10.1111/jgh.16056
[1251] Deep-learning system for real-time differentiation between Crohn's disease, intestinal Behçet's disease, and intestinal tuberculosis. — https://doi.org/10.1111/jgh.15433
[1256] The Patients' Beliefs Regarding the Role of Food, Mucosal Trauma, Menstruation, and Psychological Stress in the Recurrence of Behçet's Disease Symptoms. — https://doi.org/10.25122/jml-2019-0153
[1271] Disseminated nocardiosis involving lung, liver, and spleen in a patient with intestinal Behçet's disease under combined immunosuppressive therapy. — https://doi.org/10.1007/s12328-025-02227-7
[1273] Severe Cutaneous Manifestation of Malignant Syphilis in a Patient with Behçet's Uveitis: A Case Report. — https://doi.org/10.1159/000539723
[1279] A case of tubulointerstitial nephritis with infiltration of neutrophils and interleukin-17-positive cells associated with Behçet's disease. — https://doi.org/10.1007/s13730-025-01075-1
[1299] Enthesitis in patients with Behçet's disease: A single-centre retrospective cohort study in Japan. — https://doi.org/10.1093/mr/roaf021
[1305] Other Iatrogenic Immunodeficiency-Associated Lymphoproliferative Disorders, Diffuse Large B-Cell Lymphoma Type, in a Patient with Behçet's Disease Treated with Cyclosporine A. — https://doi.org/10.1159/000510362
[1317] HLA-B51 Positivity Correlates With Symptom Completeness From Recurrent Aphthous Stomatitis to Complete Behçet's Disease. — https://doi.org/10.1111/1346-8138.17748
[1333] Asprosin as a Potential Link Between Vascular Inflammation and Disease Activity in Behçet's Disease. — https://doi.org/10.1002/jcla.70216
[1345] Continuous evolution of clinical phenotype in 578 Japanese patients with Behçet's disease: a retrospective observational study. — https://doi.org/10.1186/s13075-016-1115-x
[1354] Development and validation of a Behçet's Disease Damage Index for adults with BD: An Explicit, Composite and Rated (ECR) tool. — https://doi.org/10.1111/1756-185x.14166
[1381] A case of Behçet's-like disease associated with trisomy 8-positive myelodysplastic syndrome carrying MEFV E148Q variant presented with periodic fever. — https://doi.org/10.1093/mrcr/rxad015
[1424] Genome-wide association analysis identifies new susceptibility loci for Behçet's disease and epistasis between HLA-B*51 and ERAP1. — https://doi.org/10.1038/ng.2520
[1437] A case of vascular Behçet's disease complicated with acute myocardial infarction due to spontaneous coronary artery dissection. — https://doi.org/10.1016/j.jccase.2020.05.016
[1444] Clinical trait-specific genetic analysis in Behçet's disease identifies novel loci associated with ocular and neurological involvement. — https://doi.org/10.1016/j.clim.2023.109657
[1445] Colchicine efficacy on oral ulcers caused by Behçet's spectrum disorders including idiopathic recurrent aphthous stomatitis, PFAPA, and Behçet's Disease. — https://doi.org/10.1007/s00431-025-06363-7
[1459] COVID-19 in patients with Behçet's disease: Outcomes and rate of Behçet's exacerbations in a retrospective cohort. — https://doi.org/10.1093/mr/roab016
[1475] Oral health is a mediator for disease severity in patients with Behçet's disease: A multiple mediation analysis study. — https://doi.org/10.1111/joor.12750
[1488] Association of long non-coding RNAs NEAT1, and MALAT1 expression and pathogenesis of Behçet's disease among Egyptian patients. — https://doi.org/10.1016/j.sjbs.2022.103344
[1496] Dense genotyping of immune-related loci implicates host responses to microbial exposure in Behçet's disease susceptibility. — https://doi.org/10.1038/ng.3786
[1555] Correlation of clinical signs and symptoms of Behçet's disease with mean platelet volume (MPV) and red cell distribution width (RDW). — https://doi.org/10.1186/s13023-020-01588-1
[1580] Myeloid sarcoma arising at the uterine cervix in a patient with intestinal Behçet's disease and concurrent myelodysplastic syndrome: A case report. — https://doi.org/10.1097/md.0000000000031559
[1590] Behçet's Disease Under Microbiotic Surveillance? A Combined Analysis of Two Cohorts of Behçet's Disease Patients. — https://doi.org/10.3389/fimmu.2020.01192
[1592] Genome-wide association studies identify IL23R-IL12RB2 and IL10 as Behçet's disease susceptibility loci. — https://doi.org/10.1038/ng.624
[1596] IL-17, IL-10, IL-6, and IFN-γ in Egyptian Behçet's disease: correlation with clinical manifestations. — https://doi.org/10.1684/ecn.2019.0421
[1602] Haploinsufficiency of A20 caused by a novel nonsense variant or entire deletion of TNFAIP3 is clinically distinct from Behçet's disease. — https://doi.org/10.1186/s13075-019-1928-5
[1642] Successful Surgical Treatment of Pediatric Intestinal Behçet's Disease with Ileocecal Ulcer Refractory to Medical Therapy: A Case Report. — https://doi.org/10.70352/scrj.cr.25-0758
[1647] Successful treatment of a refractory intestinal Behcet's disease with an oncology history by Vedolizumab: a case report and literature review. — https://doi.org/10.3389/fimmu.2023.1205046
[1654] Pilot case-control study to explore the value of intestinal ultrasound in the differentiation of two common diseases involving the ileocecal region: intestinal Behçet's disease and Crohn's disease. — https://doi.org/10.21037/qims-20-1334
[1655] The Efficacy of Medium- to Long-term Anti-TNF-α Antibody-based Maintenance Therapy in Behçet's Disease Patients with Intestinal Lesions. — https://doi.org/10.2169/internalmedicine.5000-20
[1721] Distribution of monocytes subpopulations in the peripheral blood from patients with Behçet's disease - Impact of disease status and colchicine use. — https://doi.org/10.1016/j.clim.2021.108854
[1744] A Clinical Audit on the Predictors for Visual Morbidity in Patients with Behçet's Disease Attending Cairo University Hospitals. — https://doi.org/10.1080/08820538.2020.1772318
[1775] Clinical Characteristics, MRI Findings, Disease Progression, and Management of Neuro-Behçet's Disease: A Retrospective Study in Lebanon. — https://doi.org/10.3390/jcm14082543
[1776] Demographics and clinical manifestations in patients with Behçet's disease: a retrospective observational study from a non-endemic country. — https://doi.org/10.1007/s00296-025-05855-x
[1778] Correlation of clinical signs and symptoms of Behçet's disease with platelet-to-lymphocyte ratio (PLR) and neutrophil-to-lymphocyte ratio (NLR). — https://doi.org/10.1007/s12026-021-09194-4
[1784] Femoral Vein Wall Thickness Measurement May Be a Distinctive Diagnostic Tool to Differentiate Behçet's Disease with Intestinal Involvement and Crohn's Disease. — https://doi.org/10.1007/s10620-020-06587-7
[1813] Behçet's Disease with Intestinal Involvement can be Distinguished from Inflammatory Bowel Diseases by Measurement of Common Femoral Vein Wall Thickness. — https://doi.org/10.31138/mjr.120824.dfh
[1815] Nanocurcumin supplementation ameliorates Behcet's disease by modulating regulatory T cells: A randomized, double-blind, placebo-controlled trial. — https://doi.org/10.1016/j.intimp.2021.108237
[1817] Upadacitinib for refractory Behçet's disease with myelodysplastic syndrome and trisomy 8/9: a case report and mechanistic insights. — https://doi.org/10.3389/fimmu.2025.1609884
[1823] Comparative Study of Two Classification Criteria Sets in Real Clinical Practice for Behçet's Disease. — https://doi.org/10.3390/jcm14155559
[1824] Measuring the Whole Wall Thickness of the Common Femoral Vein as a Distinctive Diagnostic Tool to Distinguish Behçet's Disease Presenting with Oral Ulcers from Recurrent Aphthous Stomatitis. — https://doi.org/10.3390/diagnostics13162705
[1829] Artificial Intelligence-Driven Proteomics Identifies Plasma Protein Signatures for Diagnosis and Stratification of Behçet's Disease. — https://doi.org/10.1002/advs.202510061
[1839] Relationship between C-reactive protein/albumin ratio and mucocutaneous symptom frequency and disease severity in Behçet's disease. — https://doi.org/10.55730/1300-0144.5803
[1863] Effectiveness and safety of adalimumab in patients with intestinal Behçet's disease: a real-world prospective observational study in South Korea. — https://doi.org/10.1186/s12876-023-03090-x
[1864] Focal vasculitic myositis as a primary manifestation of Behçet's disease: a case series of 10 Korean patients in a locomotive medicine clinic. — https://doi.org/10.1093/rheumatology/keab053
[1887] Musculoskeletal manifestations in a cohort of Behçet's disease patients and their impact on health-related quality of life. — https://doi.org/10.1007/s11739-025-03903-9
[2013] Influence of gender on Behçet's disease phenotype and irreversible organ damage: Data from the International AIDA Network Behçet's Disease Registry. — https://doi.org/10.1016/j.jbspin.2024.105819
[2035] Evaluation of pro-atherogenic lipid profile and high atherohenic indexes in patients with Behçet's disease: A case-control study. — https://doi.org/10.1111/jocd.15647
[2038] The safety and efficacy of TNF inhibitors in patients with Behçet's disease: Retrospective study from eastern Turkey. — https://doi.org/10.1016/j.clim.2024.110239
[2100] Evaluation of Left Ventricular, Left Atrial, and Right Ventricular Function in Patients With Behçet's Disease by Strain Echocardiography. — https://doi.org/10.1111/echo.70397
[2102] Efficacy and safety of apremilast and its impact on serum cytokine levels in patients with Behçet's disease. — https://doi.org/10.1111/dth.15616
[2108] Behçet's disease as a causative factor of cerebral venous sinus thrombosis: subgroup analysis of data from the VENOST study. — https://doi.org/10.1093/rheumatology/key153
[2113] Earlier and more aggressive treatment with biologics may prevent relapses and further new organ involvement in Behçet's disease. — https://doi.org/10.1016/j.clim.2023.109263
[2135] THBS1 macrophages promote aortic aneurysm formation by orchestrating an inflammatory and smooth muscle cell phenotypic transition program in vascular Behçet's disease. — https://doi.org/10.1186/s12916-025-04434-y
[2139] Relation of Social Participation Restrictions with Worsening Quality of Life in Japanese Patients with Behçet's Disease: The 36-item Short Form Survey. — https://doi.org/10.31662/jmaj.2024-0054
[2144] Identification of multiple independent susceptibility loci in the HLA region in Behçet's disease. — https://doi.org/10.1038/ng.2551
[2156] Genome-wide association study identifies variants in the MHC class I, IL10, and IL23R-IL12RB2 regions associated with Behçet's disease. — https://doi.org/10.1038/ng.625
[2164] Development of an Arabic version of the Behçet's Disease Current Activity Form (Ar-BDCAF): cross-cultural adaptation and validation initiative in Egypt. — https://doi.org/10.1007/s10067-021-05817-1
[2170] Pathergy test with a 23G needle with and without self-saliva in patients with Behçet's disease, recurrent aphthous stomatitis and control group compared to the 20G test. — https://doi.org/10.5114/reum.2021.110567
[2210] A Comprehensive Patient-Centric Analysis of Disease Burden, Treatment Challenges, and Unmet Needs in Behçet's Disease: Insights from a Large Cohort Study. — https://doi.org/10.3390/medicina62010220
[2211] Computed tomography pulmonary angiography (CTPA) in Behçet's disease patients: a remarkable gender gap and time to refine the treatment strategy. — https://doi.org/10.1007/s10067-021-05991-2
[2248] Comparative analysis of optical coherence tomography angiography (OCTA) results between Behçet's disease patients and a healthy control group. — https://doi.org/10.1007/s10067-024-06874-y
[2251] TNF inhibitors target a mevalonate metabolite/TRPM2/calcium signaling axis in neutrophils to dampen vasculitis in Behçet's disease. — https://doi.org/10.1038/s41467-024-53528-3
[2304] Effect of prophylactic benzathine penicillin on mucocutaneous symptoms of Behçet's disease. — https://doi.org/10.1159/000246336
[2306] Long Term Outcomes and Potential Risk Factors for Endovascular Repair of Aortic Pseudoaneurysms in Vascular Behçet's Disease. — https://doi.org/10.1016/j.ejvs.2023.04.009
[2338] Myelodysplastic Syndrome With Trisomy 8 and Behçet Disease-Like Intestinal and Upper Gastrointestinal Involvement. — https://doi.org/10.1002/ccr3.70575
[2343] Successful upadacitinib treatment in anti-TNF-refractory intestinal Behçet's disease: a case report and literature review. — https://doi.org/10.3389/fmed.2026.1783949
[2379] The use of non-TNF targeted biologics in Behçet's Disease: Real-life data from the International AIDA Network Behçet's Disease Registry. — https://doi.org/10.1093/rheumatology/keag086
[2380] Clinical features and prognosis of patients with gastrointestinal Behçet's disease-like syndrome and myelodysplastic syndrome with and without trisomy 8. — https://doi.org/10.1016/j.semarthrit.2022.152039
[2398] Proteomic analysis-based discovery of a novel biomarker that differentiates intestinal Behçet's disease from Crohn's disease. — https://doi.org/10.1038/s41598-021-90250-2
[2419] Autologous Hematopoietic Stem Cell Transplantation for Behçet's Disease: A Retrospective Survey of Patients Treated in Europe, on Behalf of the Autoimmune Diseases Working Party of the European Society for Blood and Marrow Transplantation. — https://doi.org/10.3389/fimmu.2021.638709
[2420] Assessment Of Circulating Endothelial Cells And Their Progenitors As Potential Biomarkers Of Disease Activity And Damage Accrual In Behçet's Syndrome. — https://doi.org/10.2147/oarrr.s225168
[2437] High Incidence of Gastrointestinal Ulceration and Cytogenetic Aberration of Trisomy 8 as Typical Features of Behçet's Disease Associated with Myelodysplastic Syndrome: A Series of 16 Consecutive Chinese Patients from the Shanghai Behçet's Disease Database and Comparison with the Literature. — https://doi.org/10.1155/2018/8535091
[2464] High levels of endothelial progenitor cells and circulating endothelial cells in patients with Behçet's disease and their relationship to disease activity. — https://doi.org/10.1590/abd1806-4841.20198169
[2526] The risk of endothelial and erectile dysfunctions in Behçet's disease: a comparative analysis of mucocutaneous and systemic patient groups. — https://doi.org/10.5114/ada.2021.108931
[2529] Risk Factors of Reoperation in Patients with Intestinal Behçet's Disease Treated by Initial Bowel Resection. — https://doi.org/10.3390/jcm13226771
[2530] Identification of an Unconventional Subpeptidome Bound to the Behçet's Disease-associated HLA-B*51:01 that is Regulated by Endoplasmic Reticulum Aminopeptidase 1 (ERAP1). — https://doi.org/10.1074/mcp.ra119.001617
[2535] Clinical features and chronology of clinical manifestations in Behçet's disease: a cohort of 2,615 patients. — https://doi.org/10.5114/reum/202818
[2545] Efficacy and safety of adalimumab in Behçet's disease-related uveitis: a multicenter retrospective observational study. — https://doi.org/10.1007/s10067-016-3480-x
[2547] Antibodies against glycoprotein 2 display diagnostic advantages over ASCA in distinguishing CD from intestinal tuberculosis and intestinal Behçet's disease. — https://doi.org/10.1038/ctg.2018.1
[2556] Quantitative changes in peripapillary, macular, and choriocapillaris microvasculature of patients with non-ocular Behçet's disease and relationship with systemic vascular involvement, an optical coherence tomography angiography study. — https://doi.org/10.1016/j.pdpdt.2022.102749
[2562] Cerebrospinal fluid level of neurofilament light chain is associated with increased disease activity in neuro-Behçet's disease. — https://doi.org/10.55730/1300-0144.5432
[2588] Atherogenic index of plasma may be strong predictor of subclinical atherosclerosis in patients with Behçet disease. — https://doi.org/10.1007/s00393-016-0141-z
[2639] Comprehensive analysis of the association between UBAC2 polymorphisms and Behçet's disease in a Japanese population. — https://doi.org/10.1038/s41598-017-00877-3
[2673] Increased T-bet/GATA-3 and ROR-γt /Foxp3 Ratios in Cerebrospinal Fluid as Potential Criteria for Definite Neuro-Behçet's Disease. — https://doi.org/10.3390/jcm11154415
[2674] Ileocecal involvement in intestinal Behçet's disease and Crohn's disease: comparison of clinicopathological and immunophenotypic features. — https://doi.org/10.1111/1751-2980.13236
[2699] Genetic Association of a Gain-of-Function IFNGR1 Polymorphism and the Intergenic Region LNCAROD/DKK1 With Behçet's Disease. — https://doi.org/10.1002/art.41637
[2730] Discordance between patient and physician global assessment of disease activity in Behçet's syndrome: a multicenter study cohort. — https://doi.org/10.1186/s13075-020-02362-1
[2736] Decreased Level of IL-37 Correlates Negatively with Inflammatory Cytokines in Cerebrospinal Fluid of Patients with Neuro-Behcet's Disease. — https://doi.org/10.22034/iji.2019.80281
[2744] Can decreased monocyte to HDL-cholesterol ratio be a marker indicating the anti-inflammatory effect of the colchicine in Behçet's disease? A preliminary study. — https://doi.org/10.1111/dth.14013
[2757] Ultrasonographic measurement of the vascular wall thickness and intima-media thickness in patients with Behçet's disease with symptoms or signs of vascular involvement: A cross-sectional study. — https://doi.org/10.46497/archrheumatol.2021.8423
[2766] Comparative risk of blindness and vision-threatening ocular comorbidities in patients with Behçet's disease versus the general population. — https://doi.org/10.1093/rheumatology/keac531
[2790] Contribution of HLA-B*51:01 and -A*26:01 to Behçet's disease and their clinical association in Thai patients. — https://doi.org/10.1111/1756-185x.13785
[2830] Sequencing-based typing of HLA-B*51 alleles and the significant association of HLA-B*5101 and -B*5108 with Behçet's disease in Greek patients. — https://doi.org/10.1034/j.1399-0039.2002.590207.x
[2855] Echocardiographic Evaluation of Coronary Microvascular Dysfunction and Biventricular Mechanics in Behçet's Disease: The Additive Value of Coronary Sinus Flow Index. — https://doi.org/10.1111/echo.70484
[2875] On the genetics of the Silk Route: association analysis of HLA, IL10, and IL23R-IL12RB2 regions with Behçet's disease in an Iranian population. — https://doi.org/10.1007/s00251-015-0841-6
[2894] Optimal Treatment Approaches to Intestinal Behçet's Disease Complicated by Myelodysplastic Syndrome: The KASID and KSBD Multicenter Study. — https://doi.org/10.3349/ymj.2023.0321
[2915] Does anticoagulation in combination with immunosuppressive therapy prevent recurrent thrombosis in Behçet's disease? — https://doi.org/10.1177/10815589241232368
[2980] Characteristics of pain and their relationship to disease activity in UK patients with Behçet's syndrome: a prospective cohort study. — https://doi.org/10.1177/20494637231198200
[3020] Significant associations of HLA-B*5101 and B*5108, and lack of association of class II alleles with Behçet's disease in Italian patients. — https://doi.org/10.1034/j.1399-0039.1999.540605.x
[3031] Clinical usefulness of fecal calprotectin in assessing disease activity and endoscopic remission in intestinal Behçet's disease. — https://doi.org/10.5217/ir.2025.00267
[3049] Safety and efficacy of infliximab in the treatment of refractory uveoretinitis in Behçet's disease: a large-scale, long-term postmarketing surveillance in Japan. — https://doi.org/10.1186/s13075-018-1793-7
[3063] Proteomics Landscape Mapping of Organ-Resolved Behçet's Disease Using In-Depth Plasma Proteomics for Identifying Hyaluronic Binding Protein 2 Expression Associated With Vascular Involvement. — https://doi.org/10.1002/art.42348
[3113] Soluble CD40L is associated with increased oxidative burst and neutrophil extracellular trap release in Behçet's disease. — https://doi.org/10.1186/s13075-017-1443-5
[3116] Pan-immune-inflammation value could be a new marker to differentiate between vascular Behçet's disease and non-vascular Behçet's disease. — https://doi.org/10.26355/eurrev_202403_35588
[3187] Human recombinant interferon alfa-2a for the treatment of Behçet's disease with sight threatening posterior or panuveitis. — https://doi.org/10.1136/bjo.87.4.423
[3201] Patient Experiences and Challenges in the Management of Autoinflammatory Diseases-Data from the International FMF & AID Global Association Survey. — https://doi.org/10.3390/jcm13051199
[3221] Mimicking Behçet's disease: GM-CSF gain of function mutation in a family suffering from a Behçet's disease-like disorder marked by extreme pathergy. — https://doi.org/10.1111/cei.13568
[3223] Interrelated modulation of endothelial function in Behcet's disease by clinical activity and corticosteroid treatment. — https://doi.org/10.1186/ar2289
[3253] Clinical presentation and treatment outcomes of arterial involvement in Behçet's disease: a single-centre experience. — https://doi.org/10.1007/s00296-021-05022-y
[3254] The Peptidome of Behçet's Disease-Associated HLA-B*51:01 Includes Two Subpeptidomes Differentially Shaped by Endoplasmic Reticulum Aminopeptidase 1. — https://doi.org/10.1002/art.39430
[3270] The Behçet's disease-associated variant of the aminopeptidase ERAP1 shapes a low-affinity HLA-B*51 peptidome by differential subpeptidome processing. — https://doi.org/10.1074/jbc.m117.789180
[3291] Long-term safety and effectiveness of adalimumab in 462 patients with intestinal Behçet’s disease: results from a large real-world observational study. — https://doi.org/10.5217/ir.2020.00013
[3362] Safety of SARS-CoV-2 vaccination in patients with Behcet's syndrome and familial Mediterranean fever: a cross-sectional comparative study on the effects of M-RNA based and inactivated vaccine. — https://doi.org/10.1007/s00296-022-05119-y
[3369] Brief Report: Drug-Free Long-Term Remission in Severe Behçet's Disease Following Withdrawal of Successful Anti-Tumor Necrosis Factor Treatment. — https://doi.org/10.1002/art.40235
[3379] Case Report: A rare pediatric case of paraneoplastic pemphigus associated with Castleman disease misdiagnosed as Behçets disease. — https://doi.org/10.3389/fped.2024.1469495
[3384] Long-Term Outcomes and Clinical Factors Associated with Conventional Therapy Failure in Intestinal Behçet's Disease: A Retrospective Cohort Study in Japan. — https://doi.org/10.1159/000548559
[3418] Efficacy and Safety of Infliximab in Intestinal Behçet's Disease: A Multicenter, Phase 3 Study (BEGIN). — https://doi.org/10.5009/gnl220278
[3430] Clinical heterogeneity and five phenotypes identified in pediatric Behçet's syndrome: a cohort study from Shanghai Behçet's syndrome database. — https://doi.org/10.1007/s12519-023-00785-9
[3454] Apremilast for oral ulcers associated with active Behçet's syndrome over 68 weeks: long-term results from a phase 3 randomised clinical trial. — https://doi.org/10.55563/clinexprheumatol/ra8ize
[3470] Significant Decline in the Incidence of Behçet's Disease in South Korea: A Nationwide Population-Based Study (2004-2017). — https://doi.org/10.1002/acr.24408
[3504] Inner Retinal Microvasculature Damage Correlates With Outer Retinal Disruption During Remission in Behçet's Posterior Uveitis by Optical Coherence Tomography Angiography. — https://doi.org/10.1167/iovs.17-23113
[3535] Regulation of NLRP3 inflammasome by zinc supplementation in Behçet's disease patients: A double-blind, randomized placebo-controlled clinical trial. — https://doi.org/10.1016/j.intimp.2022.108825
[3633] Tocilizumab in the treatment of severe and refractory parenchymal neuro-Behçet's syndrome: case series and literature review. — https://doi.org/10.1177/1759720x20971908
[3634] Accrual of organ damage in Behçet's syndrome: trajectory, associated factors, and impact on patients' quality of life over a 2-year prospective follow-up study. — https://doi.org/10.1186/s13075-022-02947-y
[3637] Characterization of autologous platelet rich plasma (PRP) and its biological effects in patients with Behçet's Disease. — https://doi.org/10.1016/j.reth.2021.08.010
[3644] The T-SPOT.TB assay used for screening and monitoring of latent tuberculosis infection in patients with Behçet's disease pre- and post-anti-TNF treatment: A retrospective study. — https://doi.org/10.1097/jcma.0000000000000071
[3723] The incidence, clinical characteristics, and outcome of COVID-19 in a prospectively followed cohort of patients with Behçet's syndrome. — https://doi.org/10.1007/s00296-021-05056-2
[3726] Immunomodulatory and clinical responses to zinc gluconate supplementation in patients with Behçet's disease: A double-blind, randomized placebo-controlled clinical trial. — https://doi.org/10.1016/j.clnu.2022.03.019
[3734] Loss-of-function mutations in TNFAIP3 leading to A20 haploinsufficiency cause an early-onset autoinflammatory disease. — https://doi.org/10.1038/ng.3459
[3747] The Presence of Uveitis Is Associated with a Sustained Response to the Interleukin (IL)-1 Inhibitors Anakinra and Canakinumab in Behçet's Disease. — https://doi.org/10.1080/09273948.2018.1511810
[3946] A two sample mendelian randomization analysis investigates causal effects between gut microbiome and immune related Vasculitis. — https://doi.org/10.1038/s41598-024-68205-0
[4046] Lactate dehydrogenase to albumin ratio as a convenient parameter for predicting the severity of Behçet disease: a cross-sectional study. — https://doi.org/10.1186/s12865-025-00706-7
[4054] Challenges in Diagnosing Non-Nasal Natural Killer/T-Cell Lymphoma Mimicking Behçet Disease: A Case Report. — https://doi.org/10.12659/ajcr.949420
[4094] Maternal and perinatal outcomes in vasculitis: a 15-year study at a Portuguese tertiary multidisciplinary centre. — https://doi.org/10.1093/rap/rkag002
[4101] Secondary Gamma-Delta T-Cell Lymphoma Not Otherwise Specified (NOS) From Chronic Immunosuppression. — https://doi.org/10.7759/cureus.14808
[4138] Combinations of immunomodulatory agents for prevention of uveitis relapse in patients with severe Behçet's disease already on corticosteroid therapy: a randomised, open-label, head-to-head trial. — https://doi.org/10.1016/s2665-9913(24)00194-2
[4150] Effect of combined colchicine-corticosteroid treatment on neutrophil/lymphocyte ratio: a predictive marker in Behçet disease activity. — https://doi.org/10.1007/s10787-020-00701-x
[4175] Development and application of an artificial intelligence-assisted endoscopic system for automatic and accurate diagnosis of colorectal ulcers. — https://doi.org/10.1007/s00384-025-05029-y
[4195] Effects of anti-SARS-CoV-2 vaccination on safety and disease exacerbation in patients with Behçet syndrome in a monocentric cohort. — https://doi.org/10.1111/1756-185x.14387
[4235] Clinical manifestations of Behçet's disease in a large cohort of Chinese patients: gender- and age-related differences. — https://doi.org/10.1007/s10067-020-05026-2
