Genital ulcer severity score and genital health quality of life in Beh?et’s disease

摘要

Background Beh?et’s Disease (BD) is a chronic auto-inflammatory, multisystem relapsing/remitting disorder of unknown aetiology. Oro-genital ulceration is a key feature of the disease and has a major impact on the patients’ quality of life. Other clinical manifestations include ocular inflammation, rheumatologic and skin involvement, while CNS and vascular complications can lead to considerable morbidity. The availability of a valid monitoring tool for BD activity is crucial in evaluating the impact of the disease on daily life activity. The aims of this study were to validate a novel tool for monitoring genital ulceration severity in BD and to assess the impact of genital ulcers on the Genital Health Quality of Life (GHQoL). Methods Genital Ulcer Severity Score (GUSS) was developed using six genital ulcer characteristics: number, size, duration, ulcer-free period, pain and site. A total of 207 BD patients were examined, (137 females: mean age?±?SD: 39.83?±?13.42 and 70 males: mean age?±?SD: 39.98?±?11.95) from the multidisciplinary Beh?et’s Centre of Excellence at Barts Health NHS Trust. GUSS was used in conjunction with Beh?et’s Disease Current Activity Form (BDCAF). Results The over-all score of GUSS showed a strong correlation with all genital ulcer characteristics, and the strongest correlation was with the pain domain (r?=?0.936; P?