Behçet's disease: evaluation of a new instrument to measure clinical activity

Bhakta, Bipin; Brennan, Paul; James, Teifi; Chamberlain, M A; Noble, B. A.; Silman, A

doi:10.1093/rheumatology/38.8.728

Cited by 233 publications

(160 citation statements)

References 8 publications

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“…Disease activity was assessed using the Behçet᾽s Disease Current Activity Form (BDCAF) score. [14] A US scan was performed on the patient and control groups in the Radiodiagnosis Department of Cairo University. The IMT measurements on each side were taken at the common carotid artery (CCA) (10 mm before and 5-10 mm cranially to the start of the bulb) and the internal carotid artery 10 mm after the flow divider.…”

Section: Methodsmentioning

confidence: 99%

Subclinical Atherosclerosis in Behçet’s Disease

et al. 2012

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Section: Methodsmentioning

confidence: 99%

Subclinical Atherosclerosis in Behçet’s Disease

et al. 2012

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“…Both unilateral and simultaneous bilateral uveitis attacks were evaluated as a single occurrence of an attack. Extraocular manifestations were recorded using a modified version of the Behçet's Disease Current Activity Form (20), which includes questions about the number of times the patient experienced fatigue, oral aphthous ulcers, genital ulcers, erythema nodosum, papulopustular skin lesions, or other BD manifestations during the past 4 weeks. Each of these manifestations was assigned a score between 0 and 4, except at week 2, when manifestations that had occurred during the past 2 weeks were assigned a score between 0 and 2.…”

Section: Methodsmentioning

confidence: 99%

Efficacy of infliximab in the treatment of uveitis that is resistant to treatment with the combination of azathioprine, cyclosporine, and corticosteroids in behçet's disease: An open‐label trial

Tuğal-Tutkun¹,

Mudun²,

Urgancioğlu³

et al. 2005

Arthritis & Rheumatism

280

151

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Objective. To investigate the effect of infliximab on the frequency of uveitis attacks and the visual prognosis in male patients with Behçet's disease in whom uveitis was resistant to combination therapy with corticosteroids, azathioprine, and cyclosporine.Methods. The study group comprised patients who were receiving combination therapy but experienced at least 2 attacks of posterior uveitis/panuveitis or retinal vasculitis during the 6-month period prior to enrollment. Infliximab infusions (5 mg/kg) were administered at weeks 0, 2, 6, and 14. Weeks 0-22 were defined as the infusion period, and weeks 23-54 were defined as the observation period. Patients continued to receive azathioprine and corticosteroids, but cyclosporine was discontinued after the screening visit. The primary outcome measures were the absence of uveitis attacks during the infusion period (remission), and the absence of uveitis attacks throughout the study period (sustained remission).Results. Thirteen patients were enrolled in the study. Thirty-two uveitis attacks involving the posterior segment occurred during the previous-treatment period. During the infusion period, 4 patients (30.8%) remained attack-free, and 9 patients had a total of 13 uveitis attacks. Ten of these attacks (76.9%) occurred at either week 14 or week 22. One of 13 patients fulfilled the definition of sustained remission, and the remaining 12 patients had a total of 36 uveitis attacks during the observation period. The mean number of uveitis attacks and daily corticosteroid doses were significantly lower during the infusion period than during the previoustreatment period or the observation period. Although potential visual acuity was regained following infliximab infusion, this beneficial effect was not preserved until week 54. None of the patients experienced a serious adverse event.Conclusion. The results of this trial suggest that infliximab is effective in suppressing the occurrence of uveitis attacks, has a corticosteroid-sparing effect, and has favorable implications for the visual prognosis of patients with resistant Behçet's uveitis.Behçet's disease (BD) is a multisystem inflammatory disorder with a chronic, relapsing course. Patients with ocular involvement typically have bilateral nongranulomatous panuveitis and retinal vasculitis that involves all elements of the retinal vasculature (1). The course of the disease is characterized by recurrent, explosive attacks of uveitis followed by spontaneous remissions. The severity and frequency of these inflammatory episodes determine the extent of permanent damage to the intraocular structures and resultant visual loss. Permanent sequelae of posterior segment involvement include optic atrophy, maculopathy, sheathing and occlusion of retinal vessels, and diffuse atrophy and gliosis of the retina. Although the course of the disease shows individual variability, male patients have a younger age at disease onset, more severe disease, and

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“…A clinical history, including medication history, was obtained and disease activity was assessed using the Leeds Behc¸et's Disease Activity Form, which gives an activity score on a 0-12-point scale. We classified patients as having an inactive/low disease activity score (DAS) (<4) or higher activity (!4) [17]. Age-and sex-matched healthy control (HC) volunteers were recruited locally and were required to be free of significant acute or chronic inflammatory disease, and not to be on medications (women taking oral contraception were not excluded).…”

Section: Subject Recruitment and Evaluationmentioning

confidence: 99%

The exaggerated inflammatory response in Behçet's syndrome: identification of dysfunctional post-transcriptional regulation of the IFN-γ/CXCL10 IP-10 pathway

Ambrose

Khan

Ravindran

et al. 2015

Clinical and Experimental Immunology

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SummaryThe mechanisms underlying the exaggerated inflammatory response in Behc¸et's syndrome (BS) remain poorly understood. We investigated the response of CD14 1 blood monocytes to interferon (IFN)-g, focusing on the chemokine CXCL10. Chemokine synthesis and release were analysed at a protein and mRNA level following stimulation with IFN-g. Findings in BS patients were compared with 25 healthy controls (HC), 15 rheumatoid arthritis (RA) and 15 systemic lupus erythematosus (SLE) disease control patients. BS monocytes produced significantly more CXCL10 protein than HC monocytes from 2 h following IFN-g stimulation, despite equivalent quantities of mRNA, suggesting more efficient translation. This was significantly more pronounced in BS with high disease activity and in those with ocular and neurological clinical manifestations. The imbalance between CXCL10 protein and mRNA expression was not observed in either RA or SLE patients, and was not seen with other chemokines studied (CXCL9, CXCL11 and CCL2). Furthermore, BS monocytes treated with an alternative stimulant (LPS) did not show abnormal tumour necrosis factor (TNF)-a release. Sucrose density gradients to segregate monocyte CXCL10 mRNA into free RNA or polysome-associated RNA showed equal proportions in BS and HC samples, suggesting that the difference between BS and HC may be due to reduced negative control of CXCL10 translation in BS at a postinitiation level. We conclude that BS monocytes have dysfunctional posttranscriptional regulation of CXCL10 mRNA, resulting in over-expression of CXCL10 protein upon IFN-g stimulation. As CXCL10 is a chemokine that recruits mononuclear cells, this abnormality may contribute to the exaggerated inflammatory responses that characterizes BS.

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Behçet's disease: evaluation of a new instrument to measure clinical activity

Abstract: Overall, this instrument has good interobserver reliability for assessing general disease activity. We therefore suggest that this proforma has a place in routine clinical monitoring of patients with BD, as well as assessing outcome in therapeutic trials.

Cited by 233 publications

References 8 publications

Subclinical Atherosclerosis in Behçet’s Disease

Subclinical Atherosclerosis in Behçet’s Disease

Efficacy of infliximab in the treatment of uveitis that is resistant to treatment with the combination of azathioprine, cyclosporine, and corticosteroids in behçet's disease: An open‐label trial

The exaggerated inflammatory response in Behçet's syndrome: identification of dysfunctional post-transcriptional regulation of the IFN-γ/CXCL10 IP-10 pathway

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