Paediatric Behçet's disease in Iran: report of 204 cases
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Published on: September 20, 2018
Farhad Shahram1, Abdolhadi Nadji2, Massoomeh Akhlaghi3
1Behçet's Disease Unit, Rheumatology Research Centre, Shariati Hospital, Tehran University of Medical Sciences, Tehran, Iran. shahramf@tums.ac.ir.
Insights
This study analyzed Iranian pediatric Behçet
Area of Science:
- Rheumatology
- Pediatrics
- Ophthalmology
Background:
- Behçet's disease (BD) is a rare multisystemic inflammatory disorder.
- Pediatric BD (PED-BD) presents unique diagnostic and management challenges.
- Understanding regional variations in PED-BD is crucial for global health insights.
Purpose of the Study:
- To characterize the clinical and demographic features of PED-BD in an Iranian cohort.
- To compare these findings with international reports on PED-BD.
- To evaluate the utility of classification criteria for PED-BD in Iran.
Main Methods:
- Retrospective analysis of 7504 Iranian BD patients.
- Inclusion of patients diagnosed with BD before age 16.
- Data collection using a 105-item protocol covering demographics, clinical, and laboratory findings.
Main Results:
- PED-BD constituted 2.7% of the cohort; mean age at onset was 10.5 years.
- Oral aphthosis (91.7%) and ocular lesions (66.2%) were most prevalent.
- Ocular lesions were more frequent and severe compared to other reports; genital ulcers and erythema nodosum were less frequent.
Conclusions:
- The clinical spectrum of Iranian PED-BD shares similarities with global data.
- Ocular involvement in Iranian PED-BD appears more frequent and severe.
- International Criteria for Behçet's Disease (ICBD) demonstrated high sensitivity (91.7%) for classifying Iranian PED-BD patients.
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.
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