Intraocular Surgery in Behcet's Disease

Behçet’s disease (BD) is a multisystemic vasculitis characterized by multiorgan involvement presenting with recurrent oral and/or genital ulcerations, skin lesions, and uveitis. Ocular involvement often occurs as remitting panuveitis and retinal vasculitis and the visual prognosis is poor if not treated properly. Relapsing uveitis attacks and chronic steroid administration during the treatment period may increase the risk of cataract and glaucoma formation. Also, recurrent uveitis may lead to severe visual compromise due to several posterior segment complications such as vitreous opacification, vitreoretinal interface changes, retinal neovascularization, cystoid macular edema, and optic atrophy. Systemic drugs including steroids, immunosuppressive, and biologic agents usually decrease the severity and frequency of the uveitis. Cataract, glaucoma, and vitreoretinal surgeries in eyes with uveitis may show some difference from the general practice. In this manuscript, approaches in anterior and posterior segment ocular surgeries in BD patients are reviewed.

2014 ◽  
Vol 2014 ◽  
pp. 1-13 ◽  
Author(s):  
Un Chul Park ◽  
Tae Wan Kim ◽  
Hyeong Gon Yu

Behçet’s disease (BD) is a chronic recurrent systemic inflammatory disorder of unknown etiology characterized by oral and genital ulcerations, skin lesions, and uveitis. The ocular involvement of BD, or Behçet’s uveitis (BU), is characterized by panuveitis or posterior uveitis with occlusive retinal vasculitis and tends to be more recurrent and sight threatening than other endogenous autoimmune uveitides, despite aggressive immunosuppression. Although pathogenesis of BD is unclear, researches have revealed that immunological aberrations may be the cornerstone of BD development. General hypothesis of BD pathogenesis is that inflammatory response is initiated by infectious agents or autoantigens in patients with predisposing genetic factors and perpetuated by both innate and acquired immunity. In addition, a network of immune mediators plays a substantial role in the inflammatory cascade. Recently, we found that the immunopathogenesis of BU is distinct from other autoimmune uveitides regarding intraocular effector cell profiles, maturation markers of dendritic cells, and the cytokine/chemokine environment. In addition, accumulating evidence indicates the involvement of Th17 cells in BD and BU. Recent studies on genetics and biologics therapies in refractory BU also support the immunological association with the pathogenesis of BU. In this review, we provide an overview of novel findings regarding the immunopathogenesis of BU.


2021 ◽  
Vol 10 (11) ◽  
pp. 2318
Author(s):  
Julie Gueudry ◽  
Mathilde Leclercq ◽  
David Saadoun ◽  
Bahram Bodaghi

Behçet’s disease (BD) is a systemic vasculitis disease of unknown origin occurring in young people, which can be venous, arterial or both, classically occlusive. Ocular involvement is particularly frequent and severe; vascular occlusion secondary to retinal vasculitis may lead to rapid and severe loss of vision. Biologics have transformed the management of intraocular inflammation. However, the diagnosis of BD is still a major challenge. In the absence of a reliable biological marker, diagnosis is based on clinical diagnostic criteria and may be delayed after the appearance of the onset sign. However, therapeutic management of BD needs to be introduced early in order to control inflammation, to preserve visual function and to limit irreversible structural damage. The aim of this review is to provide current data on how innovations in clinical evaluation, investigations and treatments were able to improve the prognosis of uveitis associated with BD.


Behçet’s Disease (BD) is a chronic multisystem vasculitis with unknown etiology, which is characterized by the triad of recurrent oral ulcers, genital ulcers, and iritis. The eye is one of the most commonly affected organs and typical ocular involvement is panuveitis with hypopyon and retinal vasculitis. The diagnosis is based on clinical evaluation including biomicroscopic and funduscopic examination. This article aims to review the clinical findings, diagnosis, and classification of BD.


Behçet’s disease (BD) was first brought to the attention of the modern medical community by Hulusi Behçet in 1937. The disease is a systemic vasculitis characterized by a recurrent aphthous ulcer, genital ulcer, uveitis, and skin lesions. It is observed more common and severe throughout the ancient Silk Road from East Asia to the Mediterranean. Ocular involvement is reported in 25-75% of patients with BD. In most cases, it may progress to blindness if left untreated. The aim of this review is to investigate the epidemiology, prevalence, and incidence of BD.


1995 ◽  
Vol 5 (3) ◽  
pp. 192-194 ◽  
Author(s):  
I. Beiran ◽  
J. Scharf ◽  
D. Dori ◽  
B. Miller

We report a case of a young man suffering from both ankylosing spondylitis and Behçet's disease in whom the character of the ocular involvement changed according to the predominant disease at a given time. When the clinical picture was one of ankylosing spondylitis, only anterior uveitis was observed, while the clinical picture of Behçet's disease occurred with panuveitis and retinal vasculitis. To the best of our knowledge, this is the first report in the English literature of two different patterns of ocular involvement in the same patient with two seronegative arthritides.


Behcet’s disease has an intractable chronic recurrent course and ocular involvement is one of the most important morbidity reasons. Systemic steroids and immunosuppressive constitute the basis of the treatment in ocular involvement. However, both the side effects and the necessity of high dosages for ocular penetration of the medications favors the local treatment options. Periocular injections (orbital floor and subtenon) in this manner are found to be effective as an adjuvant in the treatment of intractable disease, reducing the systemic dosage and preventing the recurrences. In the intravitreal route, the most commonly used is triamcinolone acetonide (IVTA) injection. With this route, local depot effect, rapid impact on the posterior segment, and retinal vasculitis, better outcomes in vitreous haze and macular edema can be achieved. However, complications like cataract and intraocular pressure (IOP) rise are commonly seen. Other options in the intravitreal route are Fluocinolone acetonide (Retisert®) and dexamethasone (Ozurdex®) implants. Ozurdex® becomes a suitable treatment option with its long-lasting effect and low side effect profile compared to IVTA. Although Retisert® seems effective with its action that might extend to 3 years, the necessity of surgery for implantation, and the high rate of cataract and glaucoma complications, it is not preferred frequently.


2020 ◽  
Author(s):  
Maryam Masoumi ◽  
Fereydoun Davatchi ◽  
Farhad Shahram ◽  
Maassoumeh Akhlaghi ◽  
Seyedeh Tahereh Faezi ◽  
...  

Abstract Background: Behçet’s disease is an autoimmune, rare, multi-system vasculitis characterized by recurrent oral and genital ulcers, uveitis, skin lesions, and arthritis. Azathioprine has been well established as an effective therapy among other immunosuppressive drugs; however, concerns remain about the safety of azathioprine. This study is conducted to determine various types and prevalence of adverse effects of azathioprine in patients with ocular Behçet’s. Methods: We carried out a cross-sectional study on 165 patients with confirmed diagnosis of Behçet’s who suffered from ocular involvement. Data concerning the different episodes of the disease, including severity, recurrence, relapse, recovery and flare up of Behçet’s disease, were collected retrospectively, as well as the azathioprine dosage and duration of consumption. Results: Of 165 enrolled patients, 103 patients (62.43%) were male and 62 patients (37.57%) were female. Adverse events of treatment with azathioprine occurred in 11 patients (6.67%). Side effects occurred in 11 patients (6.67%) taking azathioprine. These side effects included elevation of liver enzyme in 4 patients (2.43%), hypersensitivity reaction in 2 patients (1.21%), leukopenia in 2 patients (1.21%), nausea in 1 patient (0.61%), skin tumor in 1 patient (0.61%) and concomitant pancreatitis and hepatitis in 1 patient (0.61%). Six of these cases discontinued and five of them tapered the dose. In addition, we observed severe leukopenia in two patients. Conclusion: Administration of azathioprine, with due attention to the signs, symptoms, and severity of the disease, results in a reduced rate of adverse effects, and resolving of the ocular involvement in Behçet’s disease patients.


2013 ◽  
Vol 94 (4) ◽  
pp. 545-548
Author(s):  
R F Khamitov ◽  
L Y Palmova ◽  
Z N Yakupova ◽  
E R Khasanova

Behçet’s disease is a chronic relapsing multisystemic vasculitis involving vessels of different diameter. The disease is characterized by involvement of skin and mucosa, eyes, gastrointestinal tract, joints, vessels, genitourinary system and neurological symptoms. According to data, male/female ratio is 2-10:1 The etiology of the disease is still unclear. Diagnosis is mainly based on thorough analysis of clinical manifestations. According to the International Study Group for Behçet’s Disease Diagnostic Criteria, combination of recurrent oral aphthosis and at least any two of the following: severe or scarring genital aphthosis, eye lesions (posterior uveitis, retinal vasculitis), skin lesions (nodular erythema, pseudofolliculitis, acne-like lesions) and positive pathergy test is needed to classify the patient as a patient with Behçet’s disease. Several clinical subtypes of Behçet’s disease are distinguished depending on predominant involvement of different organs and systems, including skin and mucosa, joints, eyes, or neurological manifestations. There are no specific laboratory tests for Behçet’s disease. The treatment approaches are variable depending on clinical manifestations. The effect of systemic corticosteroids, colchicine, azathioprine, chlorambucil, cyclophosphamide, sulfasalazine, methotrexate, cyclosporine, infliximab was proven. All the abovementioned requires the good clinical knowledge on Behçet’s disease from practicing doctors of different specialties, particularly - physicians. A case of highly active acute Behçet’s disease with nose, pharynx, eye and oral cavity mucosa, scrotum skin involvement is presented in the article. Questions of differential diagnosis are reviewed, specific treatment program with efficacy analysis is presented.


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