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IntroductionBehçet’s disease (BD) is a rare complex multisystem inflammatory condition characterized by the triad of symptoms: aphthous ulcers of the mouth and genitalia and uveitis.Case reportWe present the case of a 31-year-old Caucasian male who had a prolonged journey from first presentation until diagnosis of BD. For 11 years, he presented symptoms affecting the oral cavity and oropharynx, with worsening odynophagia and dysphagia and ultimately, development of stridor. Flexible laryngoscopy showed significant laryngopharyngeal ulceration and scarring. Treatment was with colchicine, corticosteroids and azathioprine and supervised by one of three newly established BD Centres of Excellence in the UK.DiscussionAlthough uncommon, ENT manifestations in patients with BD should be taken into consideration to allow for early recognition and treatment of what can become a life-threatening condition. In such situations, early referral to a BD Centre of Excellence is essential to provide confirmation of diagnosis and supervision of treatment.

Background:Behçet’s Disease(BD) is a rare chronic autoinflammatory condition that can lead to irreversible organ damage. The potential for multi-organ involvement and fluctuating activity highlights the need to perform a careful and systematic assessment of disease activity that is sensitive to change. Several disease activity tools have been used in both daily practice and clinical trials, yet there is no published data comparing the clinical utility of different tools in informing changes to therapy.Objectives:To compare the utility of two major activity scores:BD Current Activity Form (BDCAF2006)1 and Birmingham Vasculitis Activity Score (BVAS)2 in predicting physician’s decision to adjust treatment (step-up/step-down) in patients with BD.Methods:A 6-month prospective observational study was performed in a cohort of patients meeting the International Criteria for Behcet’s Disease(ICBD),at the National Centre for BD in Liverpool,UK. Participants were described for their demographics, clinical manifestations and treatment plan.BVAS and BDCAF2006 activity scores were completed for each patient at evaluation.The outcome of interest was treatment change which was classified as ‘step-up’ or ‘step-down’, reflecting escalation or de-escalation in treatment (dosage adjustment or adding new immunosuppressant), respectively. We assessed the association between BVAS and BDCAF scores and step-up/step-down treatment using Spearman rank correlation and multivariate logistic regressions, adjusting for gender, age and patient’s perception of disease activity on visual analogue scale(VAS).Odds ratios(OR) and 95% confidence intervals were calculated. Data analysis was conducted in Microsoft Excel,SPSS 2.0 and STATA.Results:Ninety-five patients met inclusion criteria: 25 males(26.3%) and 70females(73.7%) with a mean age at diagnosis of 32.7years(±11.3 SD). HLAB51 was positive in 11/51 cases(11.6%).The most frequent clinical manifestations were oral ulcerations(100%), genital ulcerations(94.7%) followed by papulo-pustular skin lesions(37.8%) arthralgia(31.6%)and headache(30.5%).Mean BVAS score(range 0-6) was 2.14(±1.8 SD) and mean BDCAF score (range 0-8) was 3.04(±1.72 SD). Both BVAS and BDCAF correlated with decision to step-up treatment (r=0.752; r=0.370, respectively).Furthermore, BVAS was more strongly associated with decision to step-up treatment than BDCAF(OR 4.25 95%CI 2.37 to 7.61; 1.51 95%CI 1.15 to 2.00, respectively). Adjusting for gender, a stronger association was observed in male participants across BVAS and BDCAF scores(OR 5.89 95%CI 1.17 to 29.63; 3.48 95%CI 1.20 to10.09,respectively). Following adjustment for patient’s perception of their disease (VAS), BVAS remained significantly associated with treatment step-up(OR 3.87 95%CI 2.08 to7.19) but not BDCAF(OR 1.30 95%CI 0.91 to1.84).Regarding different clinical manifestations,the BVAS mucocutaneous and ocular activity showed a significant odds ratio for step-up therapy(OR=5.78, CI:1.49-22.15; and OR=4.2,CI: 2.26-7.83).Conclusion:BVAS can be a useful tool to asses BD ...

Background. Behçet’s disease is a rare type of vasculitis. Validated activity and damage scores were developed for vasculitis patients in order to allow a better way to evaluate disease activity and decide treatment plans. Objective. The main objective was to compare two vasculitis activity scores applied to a group of patients diagnosed with Behçet’s disease and establish correlations between them, damage and the need for immunosuppressive therapy. The secondary objective was to evaluate the connection between damage progression, classical immunosuppressant therapy and long-term cortisone use. Methods. A study was performed on a cohort of patients diagnosed with Behçet’s disease according The International Criteria for Behçet‘s Disease (ICBD) under surveillance in one Rheumatology Centre, from a non-endemic area. Vasculitis activity and damage scores were calculated for each patient. Results. 20 patients were included in the study, with a mean age of 35.7 years ± 10.5 years standard deviation(SD), 14 (70%) under the age of 40, with a male predominance 60% (12 patients). All patients presented active disease at the time of the diagnosis. Spearman’s rank correlation coefficient between BVAS v3 and BDCAF was strong r = 0.862 and statistically significant p < 0.001. The outcome analysis after remission was calculated and rank correlation coefficient between VDI, and both BVASv3 and BDCAF was moderate (VDI-BVASv3 r = 0.747, p < 0.001, VDI - BDCAF r = 0.795, p < 0.001). As for immunosuppression induction decision and activity scores, the correlation coefficient was moderate (r = 0.734 for BVASv3, r = 0.647 for BDCAF) with p < 0.001. There was a moderate correlation between immunosuppressive treatment and VDI (r = 0.700, p < 0.001). The cause of damage (i.e. vasculitis vs. treatment) is not taken into consideration when we calculate VDI. Data analysis showed the presence of mild correlation and no statistical impact between cyclophosphamide treatment duration and damage calculated as VDI (r = 0.474, p = 0.36). In contrast, when rank correlation coefficient between cortisone therapy and VDI was calculated, a moderate statistical impact was observed (r = 0.609, p < 0.001). Conclusions. An objective assessment of disease’s activity can be obtained using disease activity indexes. A moderate to strong correlation was obtained between activity indexes, immunosuppressive treatment initiation and damage progression. Comparing the two activity indexes, it resulted that: BVASv3 correlates stronger with the need for immunosuppressive treatment and both of them are equally able to anticipate damage. Damage progression was correlated stronger with long-term cortisone use, rather than immunosuppressive therapy.

Background:There is a pressing need to understand more about the psychological experience in patients with Behçet’s Disease (BD). The presence of poor wellbeing, low mood and anxiety may affect the ability to both manage and cope with this complex disorder and also impact upon the perception and experience of disease activity1,2,3.Objectives:Our aim was to investigate the relationship between patient rating of disease activity and their psychological experience (anxiety, low mood, illness perception and coping style) in a UK Centre of Excellence for BD.Methods:This study had full UK research ethics approval.A 12-month prospective observational study of a cohort of patients diagnosed with BD according to the International Criteria for BD(ICBD) under surveillance in the BD Centre of Excellence Liverpool, UK. All patients that agreed to a psychological evaluation were included. Participants were described for demographics and clinical presentation. BD Current Activity Form 2006 and a clinician’s and patient’s visual analogue scale (VAS) were recorded. Participants completed validated self-reported measures: Brief COPE, IPQ-R, PHQ-9, GAD-7 and WEMWBS. Spearman Rank correlations, multivariate regression analysis and mediation analysis were used to investigate the relationship between patient perception of disease activity, coping mechanisms and mood. For data analysis Excel and SPSS were used.Results:86 patients were selected (24 males, 68 females; mean age at time of evaluation 43.58 years ±11.8 SD; mean disease duration 9.76years ±10.01). The correlation between Clinician’s VAS and Patient’s VAS was strong 0.754 (p<0.005). Strong correlations were also observed between Patient’s VAS and low mood (PHQ-9) r=0.533 (p<0.005), anxiety (GAD-7) r=0.433(p<0.05), illness perception- emotional representation (IPQ-R) r=0.377 (p<0.05) and emotion-focused coping (COPE) r=0.320 (p<0.05).The results of the mediation analysis indicated that patient’s perception of disease activity was a significant predictor for Dysfunctional Coping Strategies (b=1.5,se=0.57, p<0.05). Dysfunctional Coping Strategies had a mediating impact on the relationship between patient perception of disease activity (VAS) and mood 0.645(CE: 0.68-1.389).Conclusion:Psychological experience plays an important part in the patient evaluation and experience of disease activity. Patient coping strategies and psychological representation of illness experience influence their perception of disease activity and their mood. This highlights the need of patients for psychological support and for improving coping mechanisms in order to obtain a better disease control and a higher quality of life.References:[1] RJ Moots, S Campbell, Is Psychological Status Related to Symptom Experience in Behçet’s Syndrome? 2014 ACR/ARHP Annual Meeting[2] IM Atay,I Erturan, A Demirdas, GB Yaman, VA Yürekli The impact of personality on quality of life and disease activity in patients with Behcet’s disease: a pilot study Compr Psychiatry. 2014 Apr;55(3):511-7[3] MA Melikoglu,M Melikoglu The rel...