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Paediatric Behcet's Disease in Iran: Report of 204 Cases Pubmed



Shahram F1 ; Nadji A2 ; Akhlaghi M1 ; Faezi ST1 ; Chamsdavatchi C3, 5 ; Shams H3, 5 ; Ghodsi SZ3, 5 ; Davatchi F1
Authors
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Authors Affiliations
  1. 1. Behcet's Disease Unit, Rheumatology Research Centre, Shariati Hospital, Tehran University of Medical Sciences, Tehran, Iran
  2. 2. Behcet's Disease Unit, Rheumatology Research Centre Shariati Hospital, Tehran University of Medical Sciences, Tehran, Iran
  3. 3. Behcet's Disease Unit, Rheumatology Research Centre, Shariati Hospital, Tehran University of Medical Sciences
  4. 4. and Department of Dermatology, Autoimmune Bullous Diseases Research Centre, Razi Hospital, Tehran University of Medical Sciences, Tehran, Iran
  5. 5. Behcet's Disease Unit, Rheumatology Research Centre, Shariati Hospital, Tehran University of Medical Sciences
  6. 6. and Department of Ophthalmology, Farabi Eye Hospital, Tehran University of Medical Sciences, Tehran, Iran

Source: Clinical and experimental rheumatology Published:2018


Abstract

OBJECTIVES: This study proposed to report the characteristics of paediatric Behcet's disease (PED-BD) in a cohort of patients from Iran's registry and compare them with different reports throughout the world. METHODS: From a cohort of 7504 Iranian patients with Behcet's disease those diagnosed before the age of 16 years were included in this study. Data were collected on a standard protocol comprising 105 items, including demographic features, type of presentation, and different clinical and laboratory findings. RESULTS: PED-BD was seen in 2.7% of patients. The male/female ratio was 1.02/1, and the mean age at onset was 10.5±3.4. Positive familial history was present in 9.9%. As a first manifestation, oral aphthosis was the most frequent (75%) followed by ocular lesions in 19.1%. The prevalence rates of various manifestations were as follows: oral aphthosis: 91.7%; genital ulcer: 42.2%; skin: 51.5% (pseudofolliculitis: 43.1%, erythema nodosum: 10.3%); ocular lesions: 66.2% (anterior uveitis 52%, posterior uveitis 58.3%, retinal vasculitis 39.7%); articular manifestations: 30.9%; neurological involvement: 4.9%; vascular involvement: 6.4% (venous 4.9%, arterial 2.5%); gastrointestinal manifestations: 5.9%; epididymo-orchitis: 8.7% (boys); high ESR (≥20): 50.8%; abnormal urine: 14.1%; positive pathergy test: 57%; HLA-B5/51: 48.7%. ICBD criteria have the highest sensitivity for the classification of PED-BD patients in Iran (91.7%). CONCLUSIONS: The clinical spectrum of PED-BD in Iran in this study was similar to that of other reports; however, genital ulcers, skin lesions (notably erythema nodosum), and gastrointestinal involvement were noticed to occur less frequently, while ocular lesions were more frequent and more severe compared to other reports.