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Objectives
Juvenile Behçet’s disease is a rare and severe disease of childhood characterized by a chronic inflammatory vasculitis. The aim of the present study is reporting demographic, clinical and therapeutic outcomes of juvenile Behçet’s disease in a tertiary center.
Methods
The retrospective study included patients who were diagnosed Behçet’s disease before 16 years. The demographic and clinical features, and diagnostic and therapeutic strategies of patients were recorded.
Results
Seventy-two patients with jBD were included in this study; 32 were male (44.4%). Thirty (41.7%) patients had BD cases in their family. We observed oral ulceration (100%), genital ulceration (68.1%), joint involvement (36.1%) and cutaneous manifestations (34.7%) as the most common clinical findings, respectively. As severe organ involvements, 20.8% ocular, 18.1% vascular and 15.3% neurologic pathologies were seen. All patients had colchicine. Corticosteroid (20.8%) was used to treat severe cases and acute attacks. Azathioprine (23.6%) was the main immunosuppressive agent and cyclophosphamide (8.3%) was applied initially for life-threatening conditions with pulse methylprednisolone.
Conclusion
In this cohort, the prevalence of genital ulceration and family history was high, and we observed less ocular involvement, a few permanent neurological morbidities and no death.
Key Points
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In the present study, there were acceptable permanent neurological involvements as morbidity and no mortality.
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It is important noticing and managing jBD in early phase in order to prevent the devastating results.
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The awareness of jBD provides timely treatment of patients.
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The positivity of family history and HLA B51 should alert the clinician about the incomplete cases.