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      Macular hole in Behçet's disease

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          Abstract

          Objective:

          To investigate the clinical features, prevalence, role of surgical intervention and the visual prognosis of macular holes (MH) in patients with Behcet's disease (BD).

          Materials and Methods:

          Retrospective study of patients with BD and MH from January 1998 to November 2008.

          Results:

          Out of 159 patients, 21 eyes of 17 patients were identified with MH. The mean age was 38.59 (range 23-61) years and the mean follow-up period was 5.1 years (range 13-164 months). The prevalence of MH was 7%. Visual acuity (VA) at the time of presentation ranged from 20/70 to hand-motion. Optical coherence tomography (OCT) findings revealed intraretinal cysts at the edge of the MH. The mean size of MH was 983.6 um; 52% had elevated edges, 43% had flat edges and only one eye (5%) was closed postoperatively. Fluorescein angiography (FA) was consistent with macular ischemia in 76% of the cases. Human leukocyte antigen (HLA) B51 association was found in 14 of the 15 patients investigated. Six patients (out of 17) underwent pars plana vitrectomy. The final VA on their last follow-up ranged from 20/70 to 2/200. Surgical intervention for MH did not result in any visual improvement as compared to non-operated eyes. One patient lost vision completely due to elevated intraocular pressure post vitrectomy and silicon oil tamponade.

          Conclusions:

          MH in patients with BD may lead to significant visual disability. Surgical intervention does not seem to have any potential beneficial effect on the VA, probably due to significant macular ischemia and sequelae from the ocular inflammation.

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          Most cited references19

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          Uveitis in Behçet disease: an analysis of 880 patients.

          We aimed to describe the demographic and clinical features, ocular manifestations, complications, visual prognosis, and treatment in a large population of Turkish patients with Behçet uveitis. We also aimed to compare visual prognosis between male and female sex and between patients who presented before and after 1990. Observational case series. A retrospective study of 880 consecutive patients (1,567 eyes) with Behçet uveitis seen at the Uveitis Service, Department of Ophthalmology, Istanbul Faculty of Medicine, Istanbul University, from 1980 to 1998. All patients met the classification criteria of the International Study Group for Behçet's Disease. Information on the patient's sex, age at onset of uveitis, ocular features, ocular complications, visual acuity, and systemic treatment was collected. Five hundred ninety-nine patients (68%) were male and 281 (32%) were female. The mean age at onset of uveitis was 28.5 years in male and 30 years in female patients. Ocular involvement was bilateral in 78.1% and unilateral in 21.9% of the patients. Panuveitis was the most common form in both sexes. Fundus lesions as well as sight-threatening complications were more common in males. At the beginning of the follow-up, potential visual acuity was 0.1 or less in 30.9% of eyes in males and 24.2% of eyes in females. Kaplan-Meier survival analysis estimated the risks of losing useful vision (>0.1) at 5 and 10 years for males and females as 21% vs 10% and 30% vs 17%, respectively. Male patients who presented in the 1990s had a significantly lower risk of losing vision compared with male patients who presented in the 1980s. Behçet uveitis starts frequently around the end of the third decade and has a male predominance. The disease is more severe and the risk of losing useful vision is higher in males than in females. However, this risk has been significantly reduced in the 1990s.
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            Close association of HLA-Bw51 with Behçet's disease.

            Frequency of HLA-A, B, and C antigens was studied in 184 patients with Behçet's disease to investigate the immunogenetically determined predisposition to this disease. A statistically significant increased incidence of HLA-B5 or Bw51 was noted in the patients as compared with the normal control subjects. No significant difference was observed in the distribution of other HLA antigens. On the basis of HLA studies, it seems likely that the susceptibility genes to Behçet's disease closely linked to HLA-Bw51 may have been spread by the old nomadic tribes or the Turks via the Silk Route.
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              Evaluation of clinical findings according to sex in 2313 Turkish patients with Behçet's disease.

              Behçet's disease is a multisystem disease featuring mucocutaneous, ocular, articular, vascular, intestinal, urogenital, and neurologic involvement and occurs with a high prevalence in the Mediterranean including Turkey. Higher incidence of severe clinical course and systemic involvement is observed in male patients. To determine the influence of sex on the clinical course of Behçet' s disease. We retrospectively evaluated the clinical findings of 2313 Behçet patients followed up at the multidisciplinary Behçet's Disease Center at Ankara University. The male/female patient ratio was 1.03. Oral aphthae was seen in all patients. In male Behçet patients, the prevalence of mucocutaneous lesions and systemic manifestations was as follows: 85.6% genital aphthae, 45.5% erythema nodosum, 59.5% papulopustular lesions, 17.5% thrombophlebitis, 38.1% ocular involvement, 11.3% articular involvement, 11.7% vascular involvement, 3.3% neurologic involvement, 1.4% gastrointestinal involvement, and 1.8% pulmonary involvement. In female Behçet patients, the prevalence of manifestations were as follows: 91% genital aphthae, 49.8% erythema nodosum, 48.3% papulopustular lesions, 3.5% thrombophlebitis, 19.8% ocular involvement, 11.8% articular involvement, 2.1% vascular involvement, 1.3% neurologic involvement, 1.4% gastrointestinal involvement, and 0.03% pulmonary involvement. Only genital aphthae and erythema nodosum were more frequent in females. On the other hand papulopustular eruptions, thrombophlebitis, ocular, neurologic, pulmonary and vascular involvement were more frequent in males. While female patients had the best prognosis, male patients had a worse overall prognosis than females.
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                Author and article information

                Journal
                Indian J Ophthalmol
                IJO
                Indian Journal of Ophthalmology
                Medknow Publications (India )
                0301-4738
                1998-3689
                Sep-Oct 2011
                : 59
                : 5
                : 359-362
                Affiliations
                [1]Uveitis Division, King Khaled Eye Specialist Hospital, Riyadh, Kingdom of Saudi Arabia
                [1 ]Department of Ophthalmology, College of Medicine, King Saud University, Riyadh, Kingdom of Saudi Arabia
                [2 ]Department of Diagnostic Imaging, King Khaled Eye Specialist Hospital, Riyadh, Kingdom of Saudi Arabia
                [3 ]Oculoplastic and Orbit Division, King Khaled Eye Specialist Hospital, Riyadh, Kingdom of Saudi Arabia
                Author notes
                Correspondence to: Dr. Hassan Al-Dhibi, Uveitis Division, King Khaled Eye Specialist Hospital, PO Box 7191, 11462 Riyadh, Kingdom of Saudi Arabia. E-mail: hdhibi@ 123456yahoo.com
                Article
                IJO-59-359
                10.4103/0301-4738.83611
                3159316
                21836340
                612dfef5-6007-4305-9dfd-972da2b750fd
                Copyright: © Indian Journal of Ophthalmology

                This is an open-access article distributed under the terms of the Creative Commons Attribution-Noncommercial-Share Alike 3.0 Unported, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                : 13 November 2009
                : 23 December 2010
                Categories
                Original Article

                Ophthalmology & Optometry
                hla-b51,behcet's disease,macular hole,optical coherence tomography
                Ophthalmology & Optometry
                hla-b51, behcet's disease, macular hole, optical coherence tomography

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