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Behcet's Disease (BD) — QSP Model References

Curated bibliography for the Behcet's Disease QSP mechanistic map, mrgsolve ODE model, and Shiny app. Organized by topic area. Total: 42 references.

1. Disease Overview & Epidemiology

  1. Yazici H, Seyahi E, Hatemi G, Yazici Y. Behçet syndrome: a contemporary view. Nat Rev Rheumatol. 2018;14(2):107-119. PubMed 29296024

  2. Zeidan MJ, Saadoun D, Garrido M, et al. Behçet's disease physiopathology: a contemporary review. Auto Immun Highlights. 2016;7(1):4. PubMed 26821879

  3. Sakane T, Takeno M, Suzuki N, Inaba G. Behçet's disease. N Engl J Med. 1999;341(17):1284-1291. PubMed 10528040

  4. Mahr A, Belarbi L, Wechsler B, et al. Population-based prevalence study of Behçet's disease: differences by ethnic origin and low variation by age at immigration. Arthritis Rheum. 2008;58(12):3951-3959. PubMed 19035501

  5. International Study Group for Behçet's Disease. Criteria for diagnosis of Behçet's disease. Lancet. 1990;335(8697):1078-1080. PubMed 1970380

2. Genetics & HLA-B51

  1. Mizuki N, Meguro A, Ota M, et al. Genome-wide association studies identify IL23R-IL12RB2 and IL10 as Behçet's disease susceptibility loci. Nat Genet. 2010;42(8):703-706. PubMed 20622879

  2. Remmers EF, Cosan F, Kirino Y, et al. Genome-wide association study identifies variants in the MHC class I, IL10, and IL23R-IL12RB2 regions associated with Behçet's disease. Nat Genet. 2010;42(8):698-702. PubMed 20622878

  3. Maldini C, Lavalley MP, Cheminant M, et al. Relationships of HLA-B51 or B5 genotype with Behçet's disease clinical characteristics: systematic review and meta-analyses of observational studies. Rheumatology (Oxford). 2012;51(5):887-900. PubMed 22241786

  4. Kirino Y, Bertsias G, Ishigatsubo Y, et al. Genome-wide association analysis identifies new susceptibility loci for Behçet's disease and epistasis between HLA-B*51 and ERAP1. Nat Genet. 2013;45(2):202-207. PubMed 23291587

3. Innate Immunity & Neutrophil Pathology

  1. Leccese P, Alpsoy E. Behçet's Disease: An Overview of Etiopathogenesis. Front Immunol. 2019;10:1067. PubMed 31143184

  2. Ceccato F, Oliviero F, Galozzi P, et al. Neutrophil functions in Behçet's disease. Clin Exp Rheumatol. 2015;33(6 Suppl 94):S105-S109. PubMed 26339938

  3. Neves FS, Moraes JC, Kowalski SC, et al. Innate immunity in Behçet's disease. Adv Exp Med Biol. 2003;528:139-142. PubMed 12664998

  4. Pineton de Chambrun M, Wechsler B, Geri G, et al. New insights into the pathogenesis of Behçet's disease. Autoimmun Rev. 2012;11(10):687-698. PubMed 22233601

  5. Apel I, Bertsias G, Boletis JN, et al. The role of NET formation in systemic autoimmune diseases. Autoimmun Rev. 2021;20(6):102817. PubMed 33766686

4. Adaptive Immunity: Th1/Th17 Axis

  1. Chi W, Zhu X, Yang P, et al. Upregulated IL-23 and IL-17 in Behçet patients with active uveitis. Invest Ophthalmol Vis Sci. 2008;49(7):3058-3064. PubMed 18502993

  2. Hamzaoui K, Hamzaoui A, Guemira F, et al. Cytokine profile in Behçet's disease patients: relationship with disease activity. Scand J Rheumatol. 2002;31(4):205-210. PubMed 12369652

  3. Sfikakis PP, Boletis JN, Tsokos GC. Rituximab anti-B-cell therapy in systemic lupus erythematosus: pointing to the future. Curr Opin Rheumatol. 2005;17(5):550-557. PubMed 16093838

  4. Yanagihori H, Oyama N, Nakamura K, Kaneko F. Role of IL-12B promoter polymorphism in Adamantiades-Behçet's disease susceptibility: an involvement of Th1 immunoreactivity against Streptococcus sanguinis antigen. J Invest Dermatol. 2006;126(7):1534-1540. PubMed 16528367

  5. Emmi G, Becatti M, Bettiol A, et al. Behçet's Syndrome as a Model of Thrombo-Inflammation: The Role of Neutrophils. Front Immunol. 2019;10:1085. PubMed 31139186

  6. Bertsias GK, Salmon JE, Boumpas DT. Therapeutic opportunities in systemic lupus erythematosus: state of the art and prospects for the new decade. Ann Rheum Dis. 2010;69(9):1603-1611. PubMed 20693281

5. Cytokine Network & Inflammasome

  1. Seoudi N, Bergmeier LA, Drobniewski F, et al. The oral mucosal and salivary microbial community of Behçet's syndrome and recurrent aphthous stomatitis. J Oral Microbiol. 2015;7:27150. PubMed 25599970

  2. Vitale A, Rigante D, Lopalco G, et al. New Therapeutic Strategies for Behçet's Syndrome. Expert Opin Investig Drugs. 2016;25(6):749-759. PubMed 27063399

  3. de Menthon M, Lavalley MP, Maldini C, et al. HLA-B51/B5 and the risk of Behçet's disease: a systematic review and meta-analysis of case-control genetic association studies. Arthritis Rheum. 2009;61(10):1287-1296. PubMed 19790126

  4. Oztas MO, Onder M, Gurer MA, et al. Serum interleukin-18 and tumour necrosis factor-alpha levels are increased in Behçet's disease. Clin Exp Dermatol. 2005;30(1):61-63. PubMed 15663502

6. Vascular Pathology & Thrombosis

  1. Seyahi E. Behçet's syndrome: How to diagnose and treat vascular involvement. Best Pract Res Clin Rheumatol. 2016;30(2):279-295. PubMed 27090631

  2. Emmi G, Bettiol A, Hatemi G, Prisco D. Behçet's syndrome. Lancet. 2021;397(10281):1311-1328. PubMed 33812493

  3. Calamia KT, Schirmer M, Melikoglu M. Major vessel involvement in Behçet's disease: an update. Curr Opin Rheumatol. 2011;23(1):24-31. PubMed 21124079

7. Clinical Trials & Treatment

  1. Hatemi G, Silman A, Bang D, et al. EULAR recommendations for the management of Behçet disease. Ann Rheum Dis. 2008;67(12):1656-1662. PubMed 18245110

  2. Hatemi G, Christensen R, Bang D, et al. 2018 update of the EULAR recommendations for the management of Behçet's syndrome. Ann Rheum Dis. 2018;77(6):808-818. PubMed 29625968

  3. Hatemi G, Melikoglu M, Tunc R, et al. Apremilast for Behçet's syndrome — a phase 2, placebo-controlled study. N Engl J Med. 2015;372(16):1510-1518. PubMed 25853758

  4. Hatemi G, Mahr A, Ishigatsubo Y, et al. Trial of Apremilast for Oral Ulcers in Behçet's Syndrome. N Engl J Med. 2019;381(20):1918-1928. PubMed 31722153

  5. Yazici Y, Hatemi G, Bodaghi B, et al. Behçet's syndrome. Nat Rev Dis Primers. 2021;7(1):67. PubMed 34593803

  6. Vitale A, Emmi G, Lopalco G, et al. Behçet's syndrome treatment: the state of the art and future perspectives. Front Med (Lausanne). 2020;7:475. PubMed 32850938

8. Colchicine Pharmacology

  1. Yurdakul S, Mat C, Tuzun Y, et al. A double-blind trial of colchicine in Behçet's syndrome. Arthritis Rheum. 2001;44(11):2686-2692. PubMed 11710724

  2. Davatchi F, Sadeghi Abdollahi B, Shams H, et al. Colchicine versus placebo in Behçet's disease: randomized, double-blind, controlled crossover trial. Mod Rheumatol. 2009;19(5):542-549. PubMed 19636658

9. Biologic Therapies (Anti-TNF, Anti-IL-1, IL-6 inhibition)

  1. Melikoglu M, Fresko I, Mat C, et al. Short-term trial of etanercept in Behçet's disease: a double blind, placebo controlled study. J Rheumatol. 2005;32(1):98-105. PubMed 15630733

  2. Saadoun D, Wechsler B, Terrada C, et al. Adalimumab in refractory uveitis associated with Behçet's disease: four-year follow-up study (ADABT trial). Rheumatology (Oxford). 2012;51(1):6-10. PubMed 21821622

  3. Vitale A, Rigante D, Caso F, et al. Inhibition of interleukin-1 by canakinumab as a successful mono-drug strategy for the treatment of refractory Behçet's disease: a case series. Dermatology. 2014;228(3):211-214. PubMed 24577264

  4. Desbois AC, Biard L, Addimanda O, et al. Efficacy of anti-IL17A therapy in severe and refractory Behçet's uveitis. Arthritis Rheumatol. 2021;73(12):2121-2131. PubMed 34170626

10. Ocular Behcet & Outcomes

  1. Bodaghi B, Wechsler B, Le Hoang P. Therapeutic approach to ocular manifestations of Behçet's disease. Rev Med Interne. 1999;20(Suppl 3):328S-333S. PubMed 10543386

  2. Ozdal PC, Ortaç S, Taskintuna I, Firat E. Posterior segment involvement in ocular Behçet's disease. Eur J Ophthalmol. 2002;12(5):424-431. PubMed 12380677

11. Pharmacokinetics & Modeling

  1. Niel E, Scherrmann JM. Colchicine today. Joint Bone Spine. 2006;73(6):672-678. PubMed 17011223

Model Parameter Sources

Parameter Value Source
Colchicine CL 17 L/h Niel & Scherrmann 2006
Colchicine Vd ~588 L (21 L/kg) Niel & Scherrmann 2006
Adalimumab t1/2 ~2 weeks Humira PI (AbbVie)
Adalimumab V1 2.8 L Humira PI
Canakinumab t1/2 ~26 days Ilaris PI (Novartis)
Prednisolone CL 8.4 L/h Clinical pharmacology
Apremilast bioavail. 73% Otezla PI (Amgen)
Apremilast t1/2 9 h Otezla PI
HLA-B51 OR 5-6x de Menthon et al. 2009
BDCAF sensitivity 3/12 threshold EULAR 2018

Compiled for QSP model development — 2026-06-17