Paediatric Behçet’s disease: a UK tertiary centre experience
Tóm tắt
There are currently limited data regarding paediatric Behçet’s disease (BD), particularly in the UK. We describe the clinical spectrum, treatment and outcome of BD, and explore the relative sensitivities of the criteria for the diagnosis of BD in a UK paediatric cohort. Single retrospective case note review of children with a clinical diagnosis of BD presenting between 1987 and 2012. Demographics, clinical features, treatment and outcomes were recorded. The sensitivities of the International Study Group (ISG) and International Criteria for BD (ICBD) criteria were explored. BD disease activity was calculated using the Behçet’s Disease Activity Index (BDAI). Forty-six patients (22 male) were identified. Median age of onset was 4.87 (0.04–15.71) years; median time to diagnosis was 3.74 (0.25–13.48) years. Clinical features were recurrent oral ulceration (97.8 %), recurrent genital ulceration (73.9 %), gastrointestinal (58.7 %), musculoskeletal (47.83 %), cutaneous (23.9 %) involvement and uveitis (2 %). Recurrent genital ulceration was more common in female patients (P = 0.044). Thirty-seven patients (80.4 %) fulfilled the ICBD criteria; only 12 patients (26.1 %) fulfilled the ISG criteria. BDAI score at diagnosis was 7/20 (0–10/20) and significantly decreased to 5/20 (0–9/20) (P < 0.0001) at latest follow-up. The commonest systemic treatment was colchicine (76.1 %); anti-TNFα treatment was reserved for severe cases (15.5 %). Paediatric BD in the UK may present very early in life, sometimes with a family history, and with a low incidence of ocular involvement. Diagnostic delay is common. The majority of our patients required systemic therapy; anti-TNFα was reserved for severe cases and has largely superseded the use of thalidomide.
Tài liệu tham khảo
Davatchi F, Shahram F, Chams-Davatchi C, Shams H, Nadji A, Akhlaghi M et al (2010) Behcet’s disease in Iran: analysis of 6500 cases. Int J Rheum Dis 13:367–373
(1990) Criteria for diagnosis of Behcet’s disease. International Study Group for Behcet’s Disease. Lancet 335:1078–80
Alpsoy E, Donmez L, Onder M, Gunasti S, Usta A, Karincaoglu Y et al (2007) Clinical features and natural course of Behcet’s disease in 661 cases: a multicentre study. Br J Dermatol 157:901–906
Borlu M, Uksal U, Ferahbas A, Evereklioglu C (2006) Clinical features of Behcet’s disease in children. Int J Dermatol 45:713–716
Calamia KT, Wilson FC, Icen M, Crowson CS, Gabriel SE, Kremers HM (2009) Epidemiology and clinical characteristics of Behcet’s disease in the US: a population-based study. Arthritis Rheum 61:600–604
de Menthon M, Lavalley MP, Maldini C, Guillevin L, Mahr A (2009) HLA-B51/B5 and the risk of Behcet’s disease: a systematic review and meta-analysis of case-control genetic association studies. Arthritis Rheum 61:1287–1296
International Team for the Revision of the International Criteria for Behcet’s D (2014) 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 28:338–347
Karincaoglu Y, Borlu M, Toker SC, Akman A, Onder M, Gunasti S et al (2008) Demographic and clinical properties of juvenile-onset Behcet’s disease: a controlled multicenter study. J Am Acad Dermatol 58:579–584
Kronborg C, Mahar PD, Kelly R (2014) Should we keep changing the diagnostic criteria for Behcet’s disease? Dermatology 228:1–4
Sarica R, Azizlerli G, Kose A, Disci R, Ovul C, Kural Z (1996) Juvenile Behcet’s disease among 1784 Turkish Behcet’s patients. Int J Dermatol 35:109–111
Sungur GK, Hazirolan D, Yalvac I, Ozer PA, Yuksel D, Vural ET et al (2009) Clinical and demographic evaluation of Behcet disease among different paediatric age groups. Br J Ophthalmol 93:83–87
Yurdakul S, Gunaydin I, Tuzun Y, Tankurt N, Pazarli H, Ozyazgan Y et al (1988) The prevalence of Behcet’s syndrome in a rural area in northern Turkey. J Rheumatol 15:820–822
Gardner-Medwin JM, Dolezalova P, Cummins C, Southwood TR (2002) Incidence of Henoch-Schonlein purpura, Kawasaki disease, and rare vasculitides in children of different ethnic origins. Lancet 360:1197–1202
Davatchi F, Abdollahi BS, Chams-Davatchi C, Shahram F, Nadji A, Shams H et al. (2013) Validation of the revised International Criteria for Behcet’s Disease (ICBD) in Iran. Clin Rheumatol
Boulter E, Brogan P (2012) Behcet’s Disease. In: Foster H, Brogan P (eds) Paediatric Rheumatology. Oxford University Press, Oxford, pp 205–208
Bhakta BB, Brennan P, James TE, Chamberlain MA, Noble BA, Silman AJ (1999) Behcet’s disease: evaluation of a new instrument to measure clinical activity. Rheumatology (Oxford) 38:728–733
Pan H CT. (2011) LMSgrowth, a Microsoft Excel add-into access growth references based on the LMS method. [cited version 2.76 January 20, 2015]; Available from: http://www.healthforallchildren.co.uk
Kari JA, Shah V, Dillon MJ (2001) Behcet’s disease in UK children: clinical features and treatment including thalidomide. Rheumatology (Oxford) 40:933–938
Kim DK, Chang SN, Bang D, Lee ES, Lee S (1994) Clinical analysis of 40 cases of childhood-onset Behcet’s disease. Pediatr Dermatol 11:95–101
Kitaichi N, Ohno S (2008) Behcet disease in children. Int Ophthalmol Clin 48:87–91
Kone-Paut I, Darce-Bello M, Shahram F, Gattorno M, Cimaz R, Ozen S et al (2011) Registries in rheumatological and musculoskeletal conditions. Paediatric Behcet’s disease: an international cohort study of 110 patients. One-year follow-up data. Rheumatology (Oxford) 50:184–188
Lang BA, Laxer RM, Thorner P, Greenberg M, Silverman ED (1990) Pediatric onset of Behcet’s syndrome with myositis: case report and literature review illustrating unusual features. Arthritis Rheum 33:418–425
Krause I, Uziel Y, Guedj D, Mukamel M, Harel L, Molad Y et al (1999) Childhood Behcet’s disease: clinical features and comparison with adult-onset disease. Rheumatology (Oxford) 38:457–462
Arida A, Fragiadaki K, Giavri E, Sfikakis PP (2011) Anti-TNF agents for Behcet’s disease: analysis of published data on 369 patients. Semin Arthritis Rheum 41:61–70
Cantarini L, Lopalco G, Caso F, Costa L, Iannone F, Lapadula G et al. (2014) Effectiveness and tuberculosis-related safety profile of interleukin-1 blocking agents in the management of Behcet’s disease. Autoimmun Rev
Yalcindag FN, Uzun A (2012) Results of interferon alpha-2a therapy in patients with Behcet’s disease. J Ocul Pharmacol Ther 28:439–443
Bernabe E, Marcenes W, Mather J, Phillips C, Fortune F (2010) Impact of Behcet’s syndrome on health-related quality of life: influence of the type and number of symptoms. Rheumatology (Oxford) 49:2165–2171