BackgroundBehçet’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).MethodsGenital 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 Behçet’s Centre of Excellence at Barts Health NHS Trust. GUSS was used in conjunction with Behçet’s Disease Current Activity Form (BDCAF).ResultsThe 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 < 0.0001). Ulcer average size and ulcer pain were the major predicting factors in GUSS (β = 0.284; β = 0.275) respectively, and P-values were significant. Multivariate regression analysis indicated that the ulcer pain, size and site are the main ulcer characteristics having an influence on the GHQoL (R2: 0.600; P < 0.0001).ConclusionsThis study established the practicality of GUSS as a severity monitoring tool for BD genital ulcers and validated its use in 207 patients. Genital ulcers of BD have a considerable impact on the patients GHQoL.Electronic supplementary materialThe online version of this article (doi:10.1186/s13023-015-0341-7) contains supplementary material, which is available to authorized users.

This study was designed to investigate the association of oral ulceration and oral health factors, together with psycho-social well-being in Behçet’s disease (BD), and to clarify the importance of psycho-social support of patients in the overall management of BD. The study comprised of a cohort of 146 BD patients (mean age ± SD = 39.65 ± 13.20) and 20 recurrent aphthous stomatitis (RAS) patients (mean age ± SD = 42.32 ± 11.32). Oral ulcer severity score (OUSS), Behçet’s disease current activities form (BDCAF), hospital anxiety and depression scale (HADS), and the work and social adjustment scale (WSAS) were investigated. Oral health risk factors were also included. The analysis of variance, regression, and factor analysis were used to scrutinise the data. Almost 73% of patients were at high caries risk in BD and RAS groups. Thirty-nine percent of BD and forty percent of RAS had a score of BPE3 (probing depth 3.5–5.5 mm). Regression analysis revealed that OUSS and WSAS had a positive impact to increase the BDCAF score in BD patients (β = 0.395, P = 0.001; β = 0.240, P = 0.019), respectively. Dental health, periodontal health, anxiety, depression, and WSAS variables had strong loadings by factor analysis based on gender and at the time of present and absent of oral ulceration. The main oral ulcer characteristics that had significant influences on the total of oral health quality of life by 68.6% were: size, duration, ulcer-free period, and pain. The results highlighted the significant influence of oral ulceration, patients’ oral health, diet, and psycho-social well-being as multi-factorial causes on increasing disease activity in BD patients.Electronic supplementary materialThe online version of this article (10.1007/s00296-018-4117-y) contains supplementary material, which is available to authorized users.

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ObjectiveThis study analyses the 2020 survey and reviews the 2009, 2014 surveys to ascertain which Behçet's symptoms, personal and family status, patients' lifestyle, and work-related outcomes impacted on Health-Related Quality of Life (HRQoL).MethodsFour hundred and fifty-nine Behçet's patients submitted an online survey/questionnaire. Patients provided information on socio-demographic characteristics, disease duration, historical and current symptoms, systemic and topical medication, health related lifestyle, work-related outcomes regarding employment status and claiming benefits and Quality of Life (QoL) measured by EQ-5D index.ResultsFour hundred and nineteen patients met the inclusion criteria, and 371 who had full data (Males: Females: Others = 84:285:2, mean–age = 41.1 ± 23.3:38 ± 13.2:40 ± 5). The main symptoms associated with patients seeking medical care were mouth ulcers 30% and genital ulcers 23%, joint 14%, and eye problems 9%. The EQ-5D index for 2009, 2014, 2020 was (mean ± SD); 0.47 ± 0.38, 0.42 ± 0.37, 0.34 ± 0.40, respectively, p &lt; 0.05. 2020 patients had the worst values of the five domains compared to 2014 and 2009. Interestingly, mobility value was the same over the 10 years of monitoring patients. Behçet's syndrome (BS) symptoms that had significant negative impact on QoL were; 2009 (arthropathy, neurological problems, pathergy reaction, and stomach/bowel symptoms), 2014 (arthropathy, headache, neurological problems, pathergy reaction, and skin lesions), 2020 (arthropathy, neurological problems, and stomach/bowel symptoms). The 2014 and 2020 surveys reported the QoL is significantly better in patients on immunosuppressant, who did sport, continued in employment and not receiving benefits.ConclusionJoints and neurological symptoms are the main symptoms which had negative impact on BS patients over the 10 years, sociodemographic (gender, age, marital, and education status), lifestyle (medication, cannabis, drinking wine, and regular exercise), employment status (employee and no career change), and accessing benefits (never claim benefit) had significant influence on patients' HRQoL.