Behçet's disease ocular attack score 24 and visual outcome in patients with Behçet's disease

Tanaka, Rie; Murata, Hiroshi; Takamoto, Masao; Ohtomo, Kazuyoshi; Okinaga, Kimiko; Yoshida, Atsushi; Kawashima, Hidetoshi; Nakahara, Hisae; Fujino, Yujiro; Kaburaki, Toshikatsu

doi:10.1136/bjophthalmol-2015-307362

Cited by 15 publications

(12 citation statements)

References 19 publications

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“…[1][2][3] BU has a higher prevalence in the Mediterranean, 4 Far and Middle Eastern countries. 5,6 In Asia, BU was reported from Japan, 7,8 Korea, 9 Taiwan, 10 China, 11 and India. 12 The prevalence of Behçet's disease in uveitis in Southeast Asia is not known.…”

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confidence: 99%

Ocular Manifestations and Visual Outcomes of Behçet’s Uveitis in a Thai population

Pathanapitoon

Kunavisarut

Saravuttikul

et al. 2017

Ocular Immunology and Inflammation

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confidence: 99%

Ocular Manifestations and Visual Outcomes of Behçet’s Uveitis in a Thai population

Pathanapitoon

Kunavisarut

Saravuttikul

et al. 2017

Ocular Immunology and Inflammation

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“…A minor disadvantage is that the authors did not evaluate the fundus based on a standard scoring system. 3 While there is evidence that Janus kinase (JAK)-mediated pathways are activated in active Behçet's syndrome (BS), 4 our study revealed that different phenotypes of BS responded variably to tofacitinib, which suggest that the role of tofacitinib in various BS phenotypes should be addressed individually due to the complexity and heterogeneity of BS. 2 Studies on intraocular inflammatory markers revealed significantly increased levels of interleukin (IL)-2, IL-8, IL-13, tumor necrosis factor α (TNFα), eotaxin and IL-1ra in aqueous humour samples from patients with BU.…”

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confidence: 80%

Response to: ‘Correspondence on ‘A pilot study of tofacitinib for refractory Behçet’s syndrome’’ by Zouet al

2021

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Response to: 'Correspondence on 'A pilot study of tofacitinib for refractory Behçet's syndrome'' by Zou et alWe thank Zou and colleagues for their interests and insightful comments 1 on our recently published work 2 and are impressed by the encouraging results of tofacitinib on patients with refractory Behçet's uveitis (BU).In this study, 5 mg two times per day tofacitinib was given to active patients with BU with prednisone reduced from 50 to 60 mg per day to 30 mg per day and withdrawal of conventional immunosuppressants (cyclosporine A or tacrolimus), which lead to treatment 'success' and 'flare' in 10 (76.9%) and 3 (23.1%) of the 13 included patients, respectively. Moreover, all the 10 patients responded to tofacitinib discontinued corticosteroid during a follow-up period between 24 and 38 months. This study provides preliminary evidence that tofacitinib might be a promising treatment for refractory BU. A minor disadvantage is that the authors did not evaluate the fundus based on a standard scoring system. 3 While there is evidence that Janus kinase (JAK)-mediated pathways are activated in active Behçet's syndrome (BS), 4 our study revealed that different phenotypes of BS responded variably to tofacitinib, which suggest that the role of tofacitinib in various BS phenotypes should be addressed individually due to the complexity and heterogeneity of BS. 2 Studies on intraocular inflammatory markers revealed significantly increased levels of interleukin (IL)-2, IL-8, IL-13, tumor necrosis factor α (TNFα), eotaxin and IL-1ra in aqueous humour samples from patients with BU. 5 Tofacitinib is a small molecule pan-JAK inhibitor, it is reasonable to infer that tofacitinib can cross the blood-ocular barriers easily to exert its anti-inflammatory effects in the eye by inhibiting multiple cytokine pathways.Several studies have reported the effectiveness of JAK inhibitors in treating non-infectious uveitis. In a mouse model of experimental autoimmune uveitis, tofacitinib significantly suppressed secretion of IFN-γ and development of disease. 6 In a retrospective clinical study, juvenile idiopathic arthritis (JIA) cases refractory to at least two biological agents (including TNFα blockers, tocilizumab, rituximab and abatacept) responded to baricitinib 4 mg/day (n=3) and tofacitinib 10 mg/day (n=1). 7 Tofacitinib was also reported to be effective in an adult JIA patient with anterior uveitis and macular edema, 8 a patient with scleritis and a patient with anterior and intermediate uveitis. 9 Currently, a few ongoing clinical trials are evaluating the role of JAK inhibitors for non-infectious uveitis (NCT03580343, NCT04088409 and NCT03207815) but none exclusively addresses BU. Thus, future studies are expected to delineate the efficacy and safety profiles of different JAK inhibitors for refractory BU, which is indeed a devastating condition for both patients and physicians.

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“…The usefulness of the BOS24 scoring system was validated in a previous retrospective study. Tanaka et al29 analyzed 50 patients with ocular BD and found that the total BOS24 score over a 5-year period was an independent factor for BCVA deterioration. Kaburaki et al17 reported that the mean BOS24 score in each ocular attack decreased from 5.8 during the baseline period to 4.0 during the 19–24 months following IFX therapy.…”

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confidence: 99%

<p>Long-term efficacy and safety of infliximab and cyclosporine combination therapy for refractory uveoretinitis in Behçet’s disease</p>

Katsuyama¹,

Kusuhara²,

Nishisho³

et al. 2019

OPTH

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This study aimed to evaluate the long-term efficacy and safety of infliximab (IFX) and cyclosporine (CsA) combination therapy for refractory uveoretinitis in Behçet's disease (BD). Patients and methods: The study involved a retrospective review of the medical records of 11 patients with Behçet's uveoretinitis refractory to conventional treatment who had been treated with IFX+CsA combination therapy. The frequency of ocular inflammatory attacks and a Behçet's disease ocular attack score 24 (BOS24) were used as indices for the evaluation of efficacy during each 6-month period prior to and following initiation of therapy. For the assessment of safety, adverse events (AEs) were recorded throughout the treatment period. Results: The patients had received IFX+CsA combination therapy for 5.6±2.3 years. The frequency of ocular attacks per 6-month period decreased markedly from 2.9±1.6 during the baseline period to 0.6±0.9 during months 1-6, 0.5±0.9 during months 7-12, 0.3±0.5 during months 13-18, 0.3±0.7 during months 19-24, and 0.0±0.0 thereafter (P=0.003). The BOS24 score per ocular attack significantly decreased from 5.2±2.4 during the baseline period to 1.5±2.1 during months 1-6, 1.7±3.1 during months 7-12, 1.6±2.9 during months 13-18, and 0.4±1.0 during months 19-24 (P=0.002). No serious AEs were observed, with the exception of urinary tract infection and cataract progression. Two patients exhibited transient elevation of the serum creatinine level, which was normalized following a reduction in the dose of CsA. Conclusion: For refractory Behçet's uveoretinitis, IFX+CsA combination therapy offers a promising treatment option as it appears to have an acceptable safety profile and can reduce the frequency and severity of ocular inflammatory attacks over a long period of time.

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Behçet's disease ocular attack score 24 and visual outcome in patients with Behçet's disease

Abstract: BOS24-5Y was found to be a significant positive prognostic index for VA deterioration in patients with ocular Behçet's disease.

Cited by 15 publications

References 19 publications

Ocular Manifestations and Visual Outcomes of Behçet’s Uveitis in a Thai population

Ocular Manifestations and Visual Outcomes of Behçet’s Uveitis in a Thai population

Response to: ‘Correspondence on ‘A pilot study of tofacitinib for refractory Behçet’s syndrome’’ by Zouet al

<p>Long-term efficacy and safety of infliximab and cyclosporine combination therapy for refractory uveoretinitis in Behçet’s disease</p>

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Behçet's disease ocular attack score 24 and visual outcome in patients with Behçet's disease

Abstract: BOS24-5Y was found to be a significant positive prognostic index for VA deterioration in patients with ocular Behçet's disease.

Cited by 15 publications

References 19 publications

Ocular Manifestations and Visual Outcomes of Behçet’s Uveitis in a Thai population

Ocular Manifestations and Visual Outcomes of Behçet’s Uveitis in a Thai population

Response to: ‘Correspondence on ‘A pilot study of tofacitinib for refractory Behçet’s syndrome’’ by Zouet al

<p>Long-term efficacy and safety of infliximab and cyclosporine combination therapy for refractory uveoretinitis in Beh&ccedil;et&rsquo;s disease</p>

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<p>Long-term efficacy and safety of infliximab and cyclosporine combination therapy for refractory uveoretinitis in Behçet’s disease</p>