Disease activity and outcomes in juvenile Behçet’s disease: 10 years’ experience of a single centre

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CER13964
2020 Vol.38, N°5 ,Suppl.127
PI 0105, PF 0111
Paediatric Rheumatology

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PMID: 33253097 [PubMed]

Received: 23/08/2020
Accepted : 09/11/2020
In Press: 26/11/2020
Published: 10/12/2020

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.