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 Table of Contents  
Year : 2018  |  Volume : 13  |  Issue : 3  |  Page : 152-153

Classification criteria of paediatric Behcet's disease: An evolving area of study

Department of Pediatrics, Christian Medical College, Vellore, Tamil Nadu, India

Date of Web Publication21-Aug-2018

Correspondence Address:
Dr. Sathish Kumar
Department of Pediatrics, Christian Medical College, Vellore, Tamil Nadu
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0973-3698.239517

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How to cite this article:
Kumar S. Classification criteria of paediatric Behcet's disease: An evolving area of study. Indian J Rheumatol 2018;13:152-3

How to cite this URL:
Kumar S. Classification criteria of paediatric Behcet's disease: An evolving area of study. Indian J Rheumatol [serial online] 2018 [cited 2022 May 28];13:152-3. Available from:

Behcet's disease (BD) is a systemic inflammatory condition characterized by autoinflammatory and vasculitic clinical features including recurrent oral aphthosis, genital ulceration, skin, eye, neurological, and vascular inflammation.[1] It is usually diagnosed between 20 and 40 years of age, but pediatric cases of BD were also reported before the age of 16 years in 4%–26% of cases.[2] Early diagnosis in children is challenging due to the insidious nature of the disease and the low sensitivity of adult criteria in the paediatric population.

There are 17 sets of diagnosis/classification criteria for BD starting from Curth's in 1946 to the revised International Criteria for BD (ICBD) in 2014. From 1990 to 2004, many studies evaluated the performance of existing diagnosis/classification criteria for BD.[3] Among all these studies, the International Study Group (ISG) criteria [4] had a high specificity for diagnosis of BD. Hence, it is widely used as classification criterion for adults. In 2014, revised International Classifications Criteria for BD (revised ICBD)[5] with variable performance was published for adults with BD.

Recently, the paediatric BD (PEDBD) classification criteria have been developed for children. A paediatric criterion for BD 2015 (PEDBD)[6] is a recently introduced classification criterion for BD in a paediatric group with promising sensitivity. The difference from revised ICBD is the exclusion of pathergy test and the weight given to each clinical feature. The new international PEDBD classification also demonstrated a higher sensitivity (91.7%) but lower specificity (42.9%) in comparison to the ISG criteria.

In this context, the present study by Chandran and Balan [7] evaluating the performance of the ISG, revised ICBD, and PEDBD 2015 is relevant. They included 17 children who were diagnosed with BD and 8 children who had symptoms that mimic BD. The sensitivity and specificity of each criterion were determined. They found that ICBD criteria have higher sensitivity than PEDBD criteria and ISG criteria have higher specificity compared to PEDBD and revised ICBD. However, it is worth noting that as they had included 8 children who mimic BD in their analysis, the results would have been altered.

Contrary to this study, Batu and et al.[8] determined the performance of different classification criteria of BD. They included 68 BD (disease onset ≤16 years; 44.1% male) and 90 control patients. The sensitivity and specificity of PEDBD/ISG criteria were 73.5%/52.9% and 97.7%/100%, respectively. They concluded that the PEDBD criteria showed better sensitivity than ISG criteria. These results show that the latest PEDBD criteria still have to be revised and its performance in different sets of patients and controls and in different ethnicity needs to be tested.

It was anticipated that the proposed PEDBD classification criteria will undergo further revision in order to include specific symptoms and signs of BD in response to new information. Such changes would be incorporated if they resulted in a demonstrable and objective improvement in homogeneity of the categories in the classification.

As the present study had involved small number of children with BD, no meaningful conclusion can be drawn. However, the study does underscore the need for revision of existing classification criteria of BD to enable early recognition of children with this condition.

  References Top

Jennette JC, Falk RJ, Bacon PA, Basu N, Cid MC, Ferrario F, et al. 2012 revised International Chapel Hill consensus conference nomenclature of vasculitides. Arthritis Rheum 2013;65:1-1.  Back to cited text no. 1
Karincaoglu Y, Borlu M, Toker SC, Akman A, Onder M, Gunasti S, et al. Demographic and clinical properties of juvenile-onset Behçet's disease: A controlled multicenter study. J Am Acad Dermatol 2008;58:579-84.  Back to cited text no. 2
Davatchi F, Sadeghi Abdollahi B, Chams-Davatchi C, Shahram F, Shams H, Nadji A, et al. The saga of diagnostic/classification criteria in Behcet's disease. Int J Rheum Dis 2015;18:594-605.  Back to cited text no. 3
Davatchi F, Assaad-Khalil S, Calamia KT, Crook JE, Sadeghi-Abdollahi B, Schirmer M, et al. The International criteria for Behcet's disease (ICBD): A collaborative study of 27 countries on the sensitivity and specificity of the new criteria. J Eur Acad Dermatol Venereol 2014;28:338-47.  Back to cited text no. 4
Criteria for diagnosis of Behçet's disease. International study group for Behçet's disease. Lancet 1990;335:1078-80.  Back to cited text no. 5
Koné-Paut I, Shahram F, Darce-Bello M, Cantarini L, Cimaz R, Gattorno M, et al. Consensus classification criteria for paediatric Behçet's disease from a prospective observational cohort: PEDBD. Ann Rheum Dis 2016;75:958-64.  Back to cited text no. 6
Chandran VS, Balan S. Comparison of the performance of three classification criteria for Behçet's Disease in a single centre cohort from South India. Indian J Rheumatol 2018;13:158-61.  Back to cited text no. 7
Batu ED, Sönmez HE, Sözeri B, Butbul Aviel Y, Bilginer Y, Özen S. The performance of different classification criteria in paediatric Behçet's disease. Clin Exp Rheumatol 2017;35 Suppl 108:119-23.  Back to cited text no. 8


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