F. DavatchiS. Assaad-KhalilK. T. CalamiaJ. E. CrookB. Sadeghi-AbdollahiM. SchirmerT. TzellosC. C. ZouboulisM. AkhlagiA. Al-DalaanZ. S. AlekberovaA. A. AliA. AltenburgE. ArromdeeM. BaltaciM. BastosS. BenamourI. Ben GhorbelA. BoyvatL. CarvalhoW. ChenE. Ben-ChetritC. Chams-DavatchiJ. A. CorreiaJ. CrespoC. DiasY. DongF. Paixão-DuarteK. ElmuntaserA. V. ElonakovJ. Graña GilA. A. HaghdoostR. M. HayaniH. HoumanA. R. IsayevaA. R. JamshidiP. KaklamanisA. KumarA. KyrgidisW. MadanatA. NadjiK. NambaS. OhnoI. OlivieriJ. Vaz PattoN. PipitoneM. V. De QueirozF. RamosC. ResendeC. M. RosaC. SalvaraniM. J. SerraF. ShahramH. ShamsK. E. SharquieM. Sliti-KhanfirT. Tribolet De AbreuC. VasconcelosJ. VedesB. WechslerY. K. ChengZ. ZhangN. ZiaeiTehran University of Medical SciencesAlexandria Medical SchoolMayo Clinic in Jacksonville, FloridaMedizinische Universitat InnsbruckStadtisches Klinikum DessauGerman Registry of Adamantiades-Behçet's DiseaseKing Saud UniversityV.A. Nasonova Research Institute of RheumatologyThe Aga Khan University HospitalMahidol UniversityHospital São TeotónioCentre Hospitalier Universitaire Ibn-RochdDepartment of Internal MedicineAnkara UniversitesiHospital Geral de Santo AntonioChang Gung UniversityHadassah University Medical CentreCentro Hospitalar e Universitario de CoimbraSao Joao HospitalPeking Union Medical CollegeFernando da Fonseca HospitalKadisia ClinicComplejo Hospitalario Universitario Juan CanalejoUniversity of BaghdadAzerbaycan Tibb UniversitetiMedical CenterAll India Institute of Medical Sciences, New DelhiJordan HospitalHokkaido University School of MedicinePotenza and Madonna Delle Grazie HospitalInstituto Portugues de ReumatologiaAzienda Ospedaliera Santa Maria Nuova di Reggio EmiliaSanta Maria Hospital, LisbonUniversity of Lisbon Faculty of Medicine, Institute of Molecular MedicineSanto António dos Capuchos HospitalHospital do Espirito SantoHospital Sousa MartinsHopital Universitaire Pitie SalpetriereGleneagles HospitalPeking University2018-11-092018-11-092014-03-01Journal of the European Academy of Dermatology and Venereology. Vol.28, No.3 (2014), 338-34714683083092699592-s2.0-84894462149https://repository.li.mahidol.ac.th/handle/123456789/34287Objective Behçet's disease (BD) is a chronic, relapsing, inflammatory vascular disease with no pathognomonic test. Low sensitivity of the currently applied International Study Group (ISG) clinical diagnostic criteria led to their reassessment. Methods An International Team for the Revision of the International Criteria for BD (from 27 countries) submitted data from 2556 clinically diagnosed BD patients and 1163 controls with BD-mimicking diseases or presenting at least one major BD sign. These were randomly divided into training and validation sets. Logistic regression, 'leave-one-country-out' cross-validation and clinical judgement were employed to develop new International Criteria for BD (ICBD) with the training data. Existing and new criteria were tested for their performance in the validation set. Results For the ICBD, ocular lesions, oral aphthosis and genital aphthosis are each assigned 2 points, while skin lesions, central nervous system involvement and vascular manifestations 1 point each. The pathergy test, when used, was assigned 1 point. A patient scoring ≥4 points is classified as having BD. In the training set, 93.9% sensitivity and 92.1% specificity were assessed compared with 81.2% sensitivity and 95.9% specificity for the ISG criteria. In the validation set, ICBD demonstrated an unbiased estimate of sensitivity of 94.8% (95% CI: 93.4-95.9%), considerably higher than that of the ISG criteria (85.0%). Specificity (90.5%, 95% CI: 87.9-92.8%) was lower than that of the ISG-criteria (96.0%), yet still reasonably high. For countries with at least 90%-of-cases and controls having a pathergy test, adding 1 point for pathergy test increased the estimate of sensitivity from 95.5% to 98.5%, while barely reducing specificity from 92.1% to 91.6%. Conclusion The new proposed criteria derived from multinational data exhibits much improved sensitivity over the ISG criteria while maintaining reasonable specificity. It is proposed that the ICBD criteria to be adopted both as a guide for diagnosis and classification of BD. © 2013 European Academy of Dermatology and Venereology.Mahidol UniversityMedicineArticleSCOPUS10.1111/jdv.12107