Peer-reviewed article
60 Childhood Behçet disease: clinical features, management and outcome of 13 cases
El Qadiry R, Nassih H, Bourrahouat A, Ait Sab I ·
Summary
Written by AI, not yet AICS's view · claude-haiku-4-5-20251001This is a clinical case series describing 13 children diagnosed with Behçet disease, a rare vasculitis affecting blood vessels. The study documents the symptoms, diagnostic findings, and treatment outcomes over a 3-year follow-up period. Most children received corticosteroids and immunosuppressive medications, with 8 achieving complete remission and 5 achieving partial remission. The authors note that the condition can cause serious complications if not treated promptly.
Key points
- All 13 children presented with recurrent aphthous stomatitis, the hallmark symptom
- Mean age at diagnosis was 9 years, with a 3-month delay between symptom onset and diagnosis
- 8 of 13 children achieved complete remission within 15 months; 5 had partial remission; 1 lost vision in one eye
- HLA-B51 was positive in 8 patients, including 3 siblings, suggesting genetic factors
- All patients were treated with corticosteroids; additional immunosuppressive agents used in subsets
Limitations
This is a small retrospective case series from a single centre with no comparison group, limiting generalisability. The report does not provide sufficient detail on how treatment decisions were made or which specific interventions were most effective for individual symptoms.
Abstract
Abstract Background Behçet disease is a rare vasculitis that involves medium and small calibre arteries. Multisystem involvement is possible. Few are the papers that provide information about its clinical course, and outcome in the pediatric population. Also, the management is mainly based on adult guidelines. Objectives To shed light on the clinical course and outcome of juvenile Behçet disease. Methods A single center retrospective descriptive study of 13 children with Bechet disease. The mean follow-up period was for 3 years. Results We had 8 boys and 5 girls. Four of our patients were consanguineous. The mean age was 9 years. The mean interval between onset and diagnosis was 3 months. All of our patients had recurrent aphthous stomatitis with a median of 6 episodes per year. The other symptoms at diagnosis were concomitant genital ulcers in 4 cases, pseudo-folliculitis in 7 cases, urticarial rush in 4 cases, severe headache in 2 patients, stroke in one patient, prolonged and/or recurrent fever in 9 patients, uveitis in 4 cases, episcleritis in one case, arthritis in 5 cases, pulmonary hemorrhage in one case, and pyoderma gangrenosum in one case. The pathergy test was performed in all the patients, and was positive in 3 of them. Diagnosis was based on the International Criteria for Behcet's Disease. Acute phase reactants (ESR and CRP) were high in all patients. Meanwhile, granulocytosis was found in 10 patients. HLA 51 was positive in 8 patients, from whom three were siblings. All our patients were on steroids, five had colchicine, seven had azathioprine, two had methotrexate, two had cyclophosphamide, three had etanercept and one had tocilizumab. Complete remission was obtained in 8 cases in a mean time of 15 months, while five children had partial remission, and one boy lost sight of his left eye after a severe retinitis. Conclusion Behcet’s disease can cause severe sequalae if not diagnosed and treated on time. More studies are needed to establish management guidelines for pediatric population.