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      Adalimumab in Treatment of Uveitis in Pediatric Behcet’s Disease–a Case Report

      case-report

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          Abstract

          Introduction:

          Behcet’s Disease (BD) is an inflammatory disease characterized by multisystemic involvement and featured by a chronic, relapsing disease course. Recurrent ocular inflammation and macular involvement may lead to severe loss of visual acuity. Prolonged use of local and systemic corticosteroids lead to numerous side effects and complications. Very often other immunosuppressive therapies are needed. Biologic agents are promising for the treatment of Behcet’s disease-associated uveitis.

          Aim:

          To report successful treatment of paediatric uveitis due to incomplete Behçet’s disease with administration of adalimumab, after failing to react to all other modalities of treatment.

          Case report:

          We present the case of a 15-year-old girl who was treated for reccurent uveitis in both eyes with developed complications on anterior and posterior ocular segment. She was earlier treated with topical and systemic steroids for uveitis, laser photocoagulation and methotrexate, however, relapses occurred inspite all treatment modalities. Patient was diagnosed with incomplete Behcet’s disease, and adalimumab therapy was initiated. Inflammation was well-controlled by adalimumab administration without need for local or systemic corticosteroids.

          Conclusion:

          Adalimumab is effective for treating children with Behcet’s disease-associated uveitis. Control of ocular inflammation in our case was achieved without need for continuing local or systemic corticosteroids, thus preventing further complications and possible significant vision loss.

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          Most cited references17

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          HLA-B51/B5 and the risk of Behçet's disease: a systematic review and meta-analysis of case-control genetic association studies.

          To quantify by meta-analysis the genetic effect of the HLA-B5 or HLA-B51 (HLA-B51/B5) allele on the risk of developing Behçet's disease (BD) and to look for potential effect modifiers. Relevant studies were identified using the PubMed Medline database and manual searches of the literature. Pooled odds ratios (ORs) and 95% confidence intervals (95% CIs) were calculated by using the random-effects model. Subgroup meta-analyses and meta-regression analyses were undertaken to investigate the effects of selected study-level parameters on the pooled OR. Heterogeneity was assessed using the I2 statistic. Pooled results were used to calculate population-attributable risks (PAR) for BD in relationship to HLA-B51/B5. A total of 4,800 patients with BD and 16,289 controls from 78 independent studies (published 1975-2007) were selected. The pooled OR of HLA-B51/B5 allele carriers to develop BD compared with noncarriers was 5.78 (95% CI 5.00-6.67), with moderate between-study heterogeneity (I2 = 61%). The subgroup analyses stratifying studies by geographic locations (Eastern Asia, Middle East/North Africa, Southern Europe, Northern/Eastern Europe) yielded consistent OR ranges (5.31-7.20), with I2 ranges of 52-70%. Univariate random-effects meta-regression indicated the percentage of male BD cases (P = 0.008) as a source of heterogeneity. The PAR within the various geographic areas were estimated at 32-52%. The strength of the association between BD and HLA-B51/B5, and its consistency across populations of various ethnicities, lends further support to this allele being a primary and causal risk determinant for BD. Variations according to sex support an interaction of this allele with BD characteristics.
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            Behçet's disease, the Silk Road and HLA-B51: historical and geographical perspectives.

            Behçet's disease (BD), also known as the Silk Road disease, is a blinding inflammatory disorder of young adults found predominantly between the Mediterranean basin and the Orient, and is strongly associated with the major histocompatibility complex (MHC) antigen HLA-B51. In this article we review the history of Behçet's disease since its first description by Hippocrates, the development of the trading routes collectively known as the Silk Road and the effect of population movement on the distribution of HLA-B51. The global distribution of this antigen among healthy control populations bears a striking similarity both to the ancient trading routes and the distribution of Behçet's disease, suggesting a genetic risk that migrated in parallel with population movement between the Mediterranean and Asia. However, certain indigenous Amerindian peoples have a high prevalence of HLA-B51 but no reported cases of BD. Furthermore, a clear genealogical relationship exists between eastern, but not central, Siberian populations with the Amerindians. Since a high level of recombination within the MHC is known to have occurred in these eastern populations before their migration into Beringia, we suggest that disruption of genetic loci in linkage disequilibria with HLA-B51 may be one reason for the absence of disease in these high HLA-B51-bearing populations. However, a contributory influence of environmental factors is not excluded by this data, and the wide variation that exists in relative risk of HLA-B51 even within Europe would support other non-genetic risk factors on the Silk Road which may be absent, or non-contributory to disease, in the Americas.
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              Behçet's disease: New insights into pathophysiology, clinical features and treatment options.

              Behçet's disease (BD) is a rare systemic vasculitis characterized by oral aphthous ulcers, genital ulcers, ocular lesions and other systemic manifestations. BD occurs most frequently in Eurasian populations along the ancient trading route known as the "Silk Road" which extends from eastern Asia to the Mediterranean basin. The causes of BD are unknown: it is believed to be due to an autoimmune process triggered by an infectious or environmental agent in genetically predisposed individuals. HLA-B51 allele located in the MHC locus, on chromosome 6p, has been the most strongly associated risk factor for BD in areas along the Old Silk Route. Herpes simplex virus-1 and Streptococcus have been postulated as possible environmental triggers of BD. T cell homeostasis perturbation, especially Th1 and Th17 expansion and decreased regulation by Tregs are now supposed to be the cornerstone of BD pathogenesis. The histology shows vasculitis that involves both arteries and veins, and vessels of any size. BD is a systemic vasculitis with significant neutrophil infiltration, endothelial cell swelling, and fibrinoid necrosis. The diagnosis of BD is only supported by clinical criteria and requires the exclusion of other diagnoses based on clinical presentation. There are no pathognomonic laboratorial findings of BD. This rare disease often leads to blindness and fatal systemic involvement. Main causes of death include major vessel disease and central nervous system involvement. Corticosteroids are commonly used to treat clinical manifestations of BD in combination with immunosuppressant drugs. Tumor necrosis factor (TNF)-blocking agents such as Infliximab, Etanercept, and Adalimumab have been reported to have success in patients with BD.
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                Author and article information

                Journal
                Mater Sociomed
                Mater Sociomed
                Materia Socio-Medica
                AVICENA, d.o.o., Sarajevo (Bosnia and Herzegovina )
                1512-7680
                1986-597X
                March 2021
                : 33
                : 1
                : 78-81
                Affiliations
                [1 ]Eye Clinic, University Clinical Center Tuzla, Bosnia and Herzegovina
                [2 ]Clinic for Children’s Diseases, University Clinical Center Tuzla, Bosnia and Herzegovina
                [3 ]Public Institution, Polyclinic with a day care hospital Doboj-Jug, Bosnia and Herzegovina
                Author notes
                Corresponding author: Meliha Halilbasic, Ass. Prof. MD, PhD. Eye Clinic University Clinical Center Tuzla, Prof. I. Pasica b.b, Tuzla, Bosnia and Herzegovina, meliha.halilbasic@ 123456gmail.com . ORCID ID: https//www.orcid.org/000-0002-4702-817X.
                Article
                MSM-33-78
                10.5455/msm.2021.33.78-81
                8116094
                447d72e6-0ace-4131-9a15-8821c37e3201
                © 2021 Meliha Halilbasic, Amra Nadarevic Vodencarevic, Almira Cosickic, Amir Halilbasic, Arnes Cabric

                This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License ( http://creativecommons.org/licenses/by-nc/4.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                : 18 February 2021
                : 20 March 2021
                Categories
                Case Report

                behcet’s disease,ophthalmology,adalimumab,pediatric uveitis

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