The International Criteria for Behçet's Disease (ICBD): A collaborative study of 27 countries on the sensitivity and specificity of the new criteria

F. Davatchi, S. Assaad-Khalil, K. T. Calamia, J. E. Crook, B. Sadeghi-Abdollahi, M. Schirmer, T. Tzellos, C. C. Zouboulis, M. Akhlagi, A. Al-Dalaan, Z. S. Alekberova, A. A. Ali, A. Altenburg, E. Arromdee, M. Baltaci, M. Bastos, S. Benamour, I. Ben Ghorbel, A. Boyvat, L. CarvalhoW. Chen, E. Ben-Chetrit, C. Chams-Davatchi, J. A. Correia, J. Crespo, C. Dias, Y. Dong, F. Paixão-Duarte, K. Elmuntaser, A. V. Elonakov, J. Graña Gil, A. A. Haghdoost, R. M. Hayani, H. Houman, A. R. Isayeva, A. R. Jamshidi, P. Kaklamanis, A. Kumar, A. Kyrgidis, W. Madanat, A. Nadji, K. Namba, S. Ohno, I. Olivieri, J. Vaz Patto, N. Pipitone, M. V. De Queiroz, F. Ramos, C. Resende, C. M. Rosa, C. Salvarani, M. J. Serra, F. Shahram, H. Shams, K. E. Sharquie, M. Sliti-Khanfir, T. Tribolet De Abreu, C. Vasconcelos, J. Vedes, B. Wechsler, Y. K. Cheng, Z. Zhang, N. Ziaei

Research output: Contribution to journalArticle

389 Citations (Scopus)

Abstract

Objective 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.

Original languageEnglish
Pages (from-to)338-347
Number of pages10
JournalJournal of the European Academy of Dermatology and Venereology
Volume28
Issue number3
DOIs
Publication statusPublished - Mar 2014

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Sensitivity and Specificity
Vascular Diseases
Blood Vessels
Central Nervous System
Logistic Models
Skin

ASJC Scopus subject areas

  • Dermatology
  • Infectious Diseases

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The International Criteria for Behçet's Disease (ICBD) : A collaborative study of 27 countries on the sensitivity and specificity of the new criteria. / Davatchi, F.; Assaad-Khalil, S.; Calamia, K. T.; Crook, J. E.; Sadeghi-Abdollahi, B.; Schirmer, M.; Tzellos, T.; Zouboulis, C. C.; Akhlagi, M.; Al-Dalaan, A.; Alekberova, Z. S.; Ali, A. A.; Altenburg, A.; Arromdee, E.; Baltaci, M.; Bastos, M.; Benamour, S.; Ben Ghorbel, I.; Boyvat, A.; Carvalho, L.; Chen, W.; Ben-Chetrit, E.; Chams-Davatchi, C.; Correia, J. A.; Crespo, J.; Dias, C.; Dong, Y.; Paixão-Duarte, F.; Elmuntaser, K.; Elonakov, A. V.; Graña Gil, J.; Haghdoost, A. A.; Hayani, R. M.; Houman, H.; Isayeva, A. R.; Jamshidi, A. R.; Kaklamanis, P.; Kumar, A.; Kyrgidis, A.; Madanat, W.; Nadji, A.; Namba, K.; Ohno, S.; Olivieri, I.; Vaz Patto, J.; Pipitone, N.; De Queiroz, M. V.; Ramos, F.; Resende, C.; Rosa, C. M.; Salvarani, C.; Serra, M. J.; Shahram, F.; Shams, H.; Sharquie, K. E.; Sliti-Khanfir, M.; Tribolet De Abreu, T.; Vasconcelos, C.; Vedes, J.; Wechsler, B.; Cheng, Y. K.; Zhang, Z.; Ziaei, N.

In: Journal of the European Academy of Dermatology and Venereology, Vol. 28, No. 3, 03.2014, p. 338-347.

Research output: Contribution to journalArticle

Davatchi, F, Assaad-Khalil, S, Calamia, KT, Crook, JE, Sadeghi-Abdollahi, B, Schirmer, M, Tzellos, T, Zouboulis, CC, Akhlagi, M, Al-Dalaan, A, Alekberova, ZS, Ali, AA, Altenburg, A, Arromdee, E, Baltaci, M, Bastos, M, Benamour, S, Ben Ghorbel, I, Boyvat, A, Carvalho, L, Chen, W, Ben-Chetrit, E, Chams-Davatchi, C, Correia, JA, Crespo, J, Dias, C, Dong, Y, Paixão-Duarte, F, Elmuntaser, K, Elonakov, AV, Graña Gil, J, Haghdoost, AA, Hayani, RM, Houman, H, Isayeva, AR, Jamshidi, AR, Kaklamanis, P, Kumar, A, Kyrgidis, A, Madanat, W, Nadji, A, Namba, K, Ohno, S, Olivieri, I, Vaz Patto, J, Pipitone, N, De Queiroz, MV, Ramos, F, Resende, C, Rosa, CM, Salvarani, C, Serra, MJ, Shahram, F, Shams, H, Sharquie, KE, Sliti-Khanfir, M, Tribolet De Abreu, T, Vasconcelos, C, Vedes, J, Wechsler, B, Cheng, YK, Zhang, Z & Ziaei, N 2014, 'The International Criteria for Behçet's Disease (ICBD): A collaborative study of 27 countries on the sensitivity and specificity of the new criteria', Journal of the European Academy of Dermatology and Venereology, vol. 28, no. 3, pp. 338-347. https://doi.org/10.1111/jdv.12107
Davatchi, F. ; Assaad-Khalil, S. ; Calamia, K. T. ; Crook, J. E. ; Sadeghi-Abdollahi, B. ; Schirmer, M. ; Tzellos, T. ; Zouboulis, C. C. ; Akhlagi, M. ; Al-Dalaan, A. ; Alekberova, Z. S. ; Ali, A. A. ; Altenburg, A. ; Arromdee, E. ; Baltaci, M. ; Bastos, M. ; Benamour, S. ; Ben Ghorbel, I. ; Boyvat, A. ; Carvalho, L. ; Chen, W. ; Ben-Chetrit, E. ; Chams-Davatchi, C. ; Correia, J. A. ; Crespo, J. ; Dias, C. ; Dong, Y. ; Paixão-Duarte, F. ; Elmuntaser, K. ; Elonakov, A. V. ; Graña Gil, J. ; Haghdoost, A. A. ; Hayani, R. M. ; Houman, H. ; Isayeva, A. R. ; Jamshidi, A. R. ; Kaklamanis, P. ; Kumar, A. ; Kyrgidis, A. ; Madanat, W. ; Nadji, A. ; Namba, K. ; Ohno, S. ; Olivieri, I. ; Vaz Patto, J. ; Pipitone, N. ; De Queiroz, M. V. ; Ramos, F. ; Resende, C. ; Rosa, C. M. ; Salvarani, C. ; Serra, M. J. ; Shahram, F. ; Shams, H. ; Sharquie, K. E. ; Sliti-Khanfir, M. ; Tribolet De Abreu, T. ; Vasconcelos, C. ; Vedes, J. ; Wechsler, B. ; Cheng, Y. K. ; Zhang, Z. ; Ziaei, N. / The International Criteria for Behçet's Disease (ICBD) : A collaborative study of 27 countries on the sensitivity and specificity of the new criteria. In: Journal of the European Academy of Dermatology and Venereology. 2014 ; Vol. 28, No. 3. pp. 338-347.
@article{7f2254870d224b1b825e76385d28fe70,
title = "The International Criteria for Beh{\cc}et's Disease (ICBD): A collaborative study of 27 countries on the sensitivity and specificity of the new criteria",
abstract = "Objective Beh{\cc}et'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.",
author = "F. Davatchi and S. Assaad-Khalil and Calamia, {K. T.} and Crook, {J. E.} and B. Sadeghi-Abdollahi and M. Schirmer and T. Tzellos and Zouboulis, {C. C.} and M. Akhlagi and A. Al-Dalaan and Alekberova, {Z. S.} and Ali, {A. A.} and A. Altenburg and E. Arromdee and M. Baltaci and M. Bastos and S. Benamour and {Ben Ghorbel}, I. and A. Boyvat and L. Carvalho and W. Chen and E. Ben-Chetrit and C. Chams-Davatchi and Correia, {J. A.} and J. Crespo and C. Dias and Y. Dong and F. Paix{\~a}o-Duarte and K. Elmuntaser and Elonakov, {A. V.} and {Gra{\~n}a Gil}, J. and Haghdoost, {A. A.} and Hayani, {R. M.} and H. Houman and Isayeva, {A. R.} and Jamshidi, {A. R.} and P. Kaklamanis and A. Kumar and A. Kyrgidis and W. Madanat and A. Nadji and K. Namba and S. Ohno and I. Olivieri and {Vaz Patto}, J. and N. Pipitone and {De Queiroz}, {M. V.} and F. Ramos and C. Resende and Rosa, {C. M.} and C. Salvarani and Serra, {M. J.} and F. Shahram and H. Shams and Sharquie, {K. E.} and M. Sliti-Khanfir and {Tribolet De Abreu}, T. and C. Vasconcelos and J. Vedes and B. Wechsler and Cheng, {Y. K.} and Z. Zhang and N. Ziaei",
year = "2014",
month = "3",
doi = "10.1111/jdv.12107",
language = "English",
volume = "28",
pages = "338--347",
journal = "Journal of the European Academy of Dermatology and Venereology",
issn = "0926-9959",
publisher = "wiley",
number = "3",

}

TY - JOUR

T1 - The International Criteria for Behçet's Disease (ICBD)

T2 - A collaborative study of 27 countries on the sensitivity and specificity of the new criteria

AU - Davatchi, F.

AU - Assaad-Khalil, S.

AU - Calamia, K. T.

AU - Crook, J. E.

AU - Sadeghi-Abdollahi, B.

AU - Schirmer, M.

AU - Tzellos, T.

AU - Zouboulis, C. C.

AU - Akhlagi, M.

AU - Al-Dalaan, A.

AU - Alekberova, Z. S.

AU - Ali, A. A.

AU - Altenburg, A.

AU - Arromdee, E.

AU - Baltaci, M.

AU - Bastos, M.

AU - Benamour, S.

AU - Ben Ghorbel, I.

AU - Boyvat, A.

AU - Carvalho, L.

AU - Chen, W.

AU - Ben-Chetrit, E.

AU - Chams-Davatchi, C.

AU - Correia, J. A.

AU - Crespo, J.

AU - Dias, C.

AU - Dong, Y.

AU - Paixão-Duarte, F.

AU - Elmuntaser, K.

AU - Elonakov, A. V.

AU - Graña Gil, J.

AU - Haghdoost, A. A.

AU - Hayani, R. M.

AU - Houman, H.

AU - Isayeva, A. R.

AU - Jamshidi, A. R.

AU - Kaklamanis, P.

AU - Kumar, A.

AU - Kyrgidis, A.

AU - Madanat, W.

AU - Nadji, A.

AU - Namba, K.

AU - Ohno, S.

AU - Olivieri, I.

AU - Vaz Patto, J.

AU - Pipitone, N.

AU - De Queiroz, M. V.

AU - Ramos, F.

AU - Resende, C.

AU - Rosa, C. M.

AU - Salvarani, C.

AU - Serra, M. J.

AU - Shahram, F.

AU - Shams, H.

AU - Sharquie, K. E.

AU - Sliti-Khanfir, M.

AU - Tribolet De Abreu, T.

AU - Vasconcelos, C.

AU - Vedes, J.

AU - Wechsler, B.

AU - Cheng, Y. K.

AU - Zhang, Z.

AU - Ziaei, N.

PY - 2014/3

Y1 - 2014/3

N2 - Objective 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.

AB - Objective 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.

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