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Juvenile Behçet's disease: a tertiary center experience
- Source :
- Clinical rheumatology. 41(1)
- Publication Year :
- 2021
-
Abstract
- 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.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.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.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 • In the present study, there were acceptable permanent neurological involvements as morbidity and no mortality. • It is important noticing and managing jBD in early phase in order to prevent the devastating results. • The awareness of jBD provides timely treatment of patients. • The positivity of family history and HLA B51 should alert the clinician about the incomplete cases.
Details
- ISSN :
- 14349949
- Volume :
- 41
- Issue :
- 1
- Database :
- OpenAIRE
- Journal :
- Clinical rheumatology
- Accession number :
- edsair.pmid..........0ef81f3379c6947530ce2eb8a412f531