Paediatric Rheumatology
Paediatric Behçet's disease in Iran: report of 204 cases
F. Shahram1, A. Nadji2, M. Akhlaghi3, S.T. Faezi4, C. Chams-Davatchi5, H. Shams6, S.Z. Ghodsi7, F. Davatchi8
- Behçet's Disease Unit, Rheumatology Research Centre, Shariati Hospital, Tehran University of Medical Sciences, Tehran, Iran. shahramf@tums.ac.ir
- Behçet's Disease Unit, Rheumatology Research Centre Shariati Hospital, Tehran University of Medical Sciences, Tehran, Iran.
- Behçet's Disease Unit, Rheumatology Research Centre, Shariati Hospital, Tehran University of Medical Sciences, Tehran, Iran.
- Behçet's Disease Unit, Rheumatology Research Centre, Shariati Hospital, Tehran University of Medical Sciences, Tehran, Iran.
- Behçet’s Disease Unit, Rheumatology Research Centre, Shariati Hospital, Tehran University of Medical Sciences; and Department of Dermatology, Autoimmune Bullous Diseases Research Centre, Razi Hospital, Tehran University of Medical Sciences, Tehran, Iran.
- Behçet’s Disease Unit, Rheumatology Research Centre, Shariati Hospital, Tehran University of Medical Sciences; and Department of Ophthalmology, Farabi Eye Hospital, Tehran University of Medical Sciences, Tehran, Iran.
- Behçet’s Disease Unit, Rheumatology Research Centre, Shariati Hospital, Tehran University of Medical Sciences; and Department of Dermatology, Autoimmune Bullous Diseases Research Centre, Razi Hospital, Tehran University of Medical Sciences, Tehran, Iran.
- Behçet's Disease Unit, Rheumatology Research Centre, Shariati Hospital, Tehran University of Medical Sciences, Tehran, Iran.
CER10965
2018 Vol.36, N°6 ,Suppl.115
PI 0135, PF 0140
Paediatric Rheumatology
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PMID: 29998839 [PubMed]
Received: 13/11/2017
Accepted : 09/03/2018
In Press: 19/06/2018
Published: 14/12/2018
Abstract
OBJECTIVES:
This study proposed to report the characteristics of paediatric Behçet’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 Behçet’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.