Deniz Gezgin Yıldırım1, Sevcan A Bakkaloğlu2, Murat Hasanreisoglu3, Necla Buyan2. 1. Department of Paediatric Rheumatology, Gazi University Faculty of Medicine, Ankara, Turkey. gezgindeniz@gmail.com. 2. Department of Paediatric Rheumatology, Gazi University Faculty of Medicine, Ankara, Turkey. 3. Department of Ophthalmology, Gazi University Faculty of Medicine, Ankara, Turkey.
Abstract
OBJECTIVES: The aims of this study were to investigate the development of new events (new clinical signs related to Behçet's disease) and to evaluate outcomes in juvenile Behçet's disease (jBD) patients over a 10-year follow-up. METHODS: We included 57 patients diagnosed with jBD according to International Behçet's Study Group (ISG) criteria and/or the International Criteria for BD (ICBD) and/or Paediatric BD (PEDBD) group criteria, followed-up between 2008 and 2018. Any new organ system involvement during follow-up was defined as an event in event-free survival analysis. RESULTS: The patients' female/male ratio was 33/24. The most prevalent clinical feature was recurrent oral aphthosis (100%), followed by musculoskeletal symptoms (63%), genital ulcers (56%), ocular manifestations (47%) and cutaneous manifestations (46%). Vascular, neurological, gastrointestinal and genitourinary manifestations were observed in 4-17% of the patients. Fifty-four (95%) cases fulfilled the ICBD, while 31 (54%) and 34 (60%) fulfilled ISG and PEDBD criteria, respectively. The median Iranian Behçet's disease dynamic activity measure (IBDDAM) score at diagnosis was 5 (range: 3-14) and decreased to 1 (range: 0-6) at the last visit. One to three events occurred in 21 (37%) cases. One fifth (19%) of these events were severe. The event-free survival rate was 95% at one year, 70% at three years and 50% at eight years. CONCLUSIONS: This study shows that with effective treatment, jBD has favourable outcome and a remarkable event-free survival. Underdiagnosed cases according to ISG and PEDBD criteria could be diagnosed using the ICBD.
OBJECTIVES: The aims of this study were to investigate the development of new events (new clinical signs related to Behçet's disease) and to evaluate outcomes in juvenile Behçet's disease (jBD) patients over a 10-year follow-up. METHODS: We included 57 patients diagnosed with jBD according to International Behçet's Study Group (ISG) criteria and/or the International Criteria for BD (ICBD) and/or Paediatric BD (PEDBD) group criteria, followed-up between 2008 and 2018. Any new organ system involvement during follow-up was defined as an event in event-free survival analysis. RESULTS: The patients' female/male ratio was 33/24. The most prevalent clinical feature was recurrent oral aphthosis (100%), followed by musculoskeletal symptoms (63%), genital ulcers (56%), ocular manifestations (47%) and cutaneous manifestations (46%). Vascular, neurological, gastrointestinal and genitourinary manifestations were observed in 4-17% of the patients. Fifty-four (95%) cases fulfilled the ICBD, while 31 (54%) and 34 (60%) fulfilled ISG and PEDBD criteria, respectively. The median Iranian Behçet's disease dynamic activity measure (IBDDAM) score at diagnosis was 5 (range: 3-14) and decreased to 1 (range: 0-6) at the last visit. One to three events occurred in 21 (37%) cases. One fifth (19%) of these events were severe. The event-free survival rate was 95% at one year, 70% at three years and 50% at eight years. CONCLUSIONS: This study shows that with effective treatment, jBD has favourable outcome and a remarkable event-free survival. Underdiagnosed cases according to ISG and PEDBD criteria could be diagnosed using the ICBD.