Behçet's disease

Beh?et's disease is a chronic, relapsing vasculitis that can affect most organ systems. The prevalence varies geographically, and the disease is more common in countries along the ancient Silk Road, including Italy, Turkey, Israel, Saudi Arabia, Iran, China, Korea and Japan. Beh?et's is more common in men than in women, and typically affects young adults. The classic finding in Beh?et's patients is the presence of recurrent mucocutaneous ulcers, and oral aphthous ulcerations are usually the initial symptom. Other manifestations include genital ulcers, skin lesions, vascular, neurological, articular, and ocular disease. The disease can affect the anterior and/or posterior segments of the eye, and the main manifestations include iridocyclitis, hypopyon, mild to moderate vitreitis, retinal vasculitis and occlusion, optic disc hyperemia, and macular edema. There is no pathognomonic laboratory test in Beh?et's disease, and the diagnosis is based in systemic and ocular clinical findings. Treatment of ocular Beh?et is based in corticosteroids and immunosuppressive agents, to suppress acute inflammation and reduce its recurrence frequency. Ocular lesions may improve with immunosuppressive therapy, but usually are not fully reversible, and generally progress over time. The prognosis of anterior uveitis is usually good, but patients with posterior lesions tend to have some degree of visual loss, even with adequate treatment.     

Cataract surgery in patients with Behçet's disease

PURPOSE: To evaluate the outcomes and complications of cataract surgery in patients with Beh?et's disease. SETTING: Department of Ophthalmology, Hacettepe University School of Medicine, Ankara, Turkey. METHODS: Thirty-three eyes of 26 patients with Beh?et's disease that had extracapsular cataract surgery between January 1993 and July 1999 were analyzed retrospectively. The mean age of the patients was 38.9 years (range 20 to 54 years). The mean postoperative follow-up was 22.9 months (range 6 to 66 months). RESULTS: Extracapsular cataract extraction (ECCE) was performed in 22 eyes with and in 6 eyes without intraocular lens (IOL) implantation. Phacoemulsification with IOL implantation was performed in 5 eyes. Postoperatively, the visual acuity was better in 29 eyes (87.8%) and was 0.5 or better in 14 eyes (42.4%). Posterior segment complications of Beh?et's disease, mainly optic atrophy and macular alterations from preoperative inflammatory episodes, restricted final acuity. No significant difference was detected in postoperative inflammation among the types of surgery; that is, ECCE, ECCE with IOL implantation, and phacoemulsification with IOL implantation. A neodymium:YAG laser posterior capsulotomy was performed in 3 cases. CONCLUSIONS: In patients with Beh?et's disease, inflammation after extracapsular surgery was mild when surgery was performed after at least 3 months of no inflammatory signs. The results show that the outcomes of extracapsular cataract surgery mainly depend on the degree of preoperative posterior segment involvement.     

Macular damage in Behçet's disease

INTRODUCTION: The macular damage in Beh?et's disease requires particular attention because of its poor prognosis. PURPOSE: The purpose of our study was to specify the frequency of macular alterations in Beh?et's disease and discuss the clinical, physiopathological and therapeutic aspects. MATERIALS AND METHODS: Our report is based on 244 patients with Beh?et's disease examined between January 1995 and December 1997. The authors studied macular alterations in detail. RESULTS: Sixty-nine patients had a maculopathy affecting 108 out of 432 eyes (24%). The mean progression of Beh?et's disease in these cases was 4 years. Visual acuity was相似文献   

Drug therapy in Behçet's disease

Beh?et's disease is one of the most difficult forms of uveitis to treat. Variety in disease presentation and severity, as well as regional differences in standard of care, demand a tailor-made approach. Anterior segment inflammation generally responds to topical corticosteroids. However, the onset of posterior segment inflammation usually requires the advancement of treatment to periocular injections and/or oral administration of corticosteroids. Cyclosporin, either alone or in combination with corticosteroids, is considered in refractory patients. Other immunosuppressive drugs, such as azathioprine and chlorambucil, may be considered in difficult cases. Finally, preliminary results suggest efficacy with the immunomodulatory agent interferon alfa, although further clinical trials are necessary to evaluate safety and efficacy.     

Macular hole in Behçet's disease

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.     

Retinal vasculitis in Behçet's disease

Beh?et's disease (BD) is a multisystemic disorder characterized by primary vasculitis of unknown etiology. We studied retinal vasculitis in 35 patients with ocular form of BD. All patients fulfilled the International Study Group criteria for BD. Mean age was 30.8 +/- 6.5 years (19-45 years, mean follow-up period 4.1 +/- 1.29 years (3-10 years. The disease occurred 3.3 times more often in men than in women (p = 0.0003). Retinal vasculitis was diagnosed in 26 (74.3%) of 35 patients with ocular form of BD. Retinal vascular changes characteristic of BD were bilateral in 96.1% cases and involved both arteries and veins. The earliest changes were detectable only by fluorescent angiography. Diffuse capillary leakage was observed in all cases during the active period of disease, involving the posterior pole. The disk and macular capillaries were involved most of all. Late staining of vasculature was observed in 68.6% patients. No ruptures of retinal vessels, retinal neovascularization, or areas of retinal nonperfusion were observed (except one case with occlusion of a branch of the central retinal vein), which are usually seen in other types of retinal vasculitis. Visual acuity was significantly decreased in patients with retinal occlusive vasculitis in comparison with patients without it (0.04 +/- 0.09 vs. 0.3 +/- 0.3, p = 0.029).     

Conjunctival ulcers in Behçet's disease

PURPOSE: To describe the occurrence of conjunctival ulcers as a manifestation of Beh?et's disease. DESIGN: Retrospective, noncomparative, interventional case series with histopathologic correlation. METHODS: Six patients who fulfilled the diagnostic criteria for Beh?et's disease and presented with painful conjunctival ulcers were included in the study. Three of these ulcers were biopsied and studied histologically and immunohistochemically. The lesions were treated with topical or subconjunctival injection of corticosteroids and, in one case, with oral indomethacin. RESULTS: Although all six patients fulfilled the diagnostic criteria for Beh?et's disease, two developed uveitis and other signs of Beh?et's disease only months to years after the appearance of the conjunctival ulcers. The 3- to 5-mm, round to oval ulcers were located in the limbal and/or bulbar conjunctiva. Histopathology revealed disrupted epithelium, infiltration of both acute and chronic inflammatory cells, and high endothelial venules. Immunohistochemical analysis of the infiltrating lymphocytes revealed primarily T-cell populations admixed with several B cells and CD68-positive histiocytes. After treatment, the conjunctival lesions invariably healed without scarring. CONCLUSIONS: In addition to the oral and genital ulceration, ulcers can also be found in the conjunctiva of patients with Beh?et's disease. Although this is a rare clinical sign, when accompanied by uveitis or orogenital ulcers, it may suggest a diagnosis of Beh?et's disease.     

Visual prognosis in Behçet's disease

In the pre-cyclosporin therapy era, BenEzra and Cohen(1) reported an appalling visual prognosis of patients with Beh?et's disease, with 74% of eyes losing useful vision six to ten years after diagnosis. This experience was in a population largely of Mediterranean origin and it has been suggested that different ethnic groups may have different rates of ocular disease progression. In addition, newer immunosuppressive agents, e.g. cyclosporin, have been widely used in the last few years in the acute management of patients with Beh?et's disease though their long term effect on the visual prognosis has not been established. The authors report the visual findings in a cohort of 28 patients of mixed ethnic extraction with Beh?et's disease and ocular involvement who have been followed-up for an average of six years. Posterior segment disease relapse was treated aggressively with high dose corticosteroids, cyclosporin, azathioprine, cyclophosphamide, or chlorambucil in varying combinations. At present, approximately 60% of patients have visual acuity of ± 6/9 in the better eye, 21% are 6/12 to 6/60, and 18% worse than 6/60. These findings, albeit relatively short term at present, are encouraging enough to continue this aggressive therapeutic management in the belief that the depressing figures in the literature reflect the past and not the present.     

Orbital inflammation associated with Behçet's disease

Herein a case of a 35‐year‐old woman with a history of Behçet's disease, who presented with swelling and redness of her right eye with increasing pain, is reported. Computed tomography and magnetic resonance imaging showed enlargement of the right lacrimal gland and contrast enhancement of the extraocular muscles. A diagnosis of orbital inflammation was made and the patient was treated with corticosteroids, with prompt resolution of symptoms and clinical signs. Orbital inflammation should be considered as an ophthalmic manifestation of Behçet's disease.     

Soluble adhesion molecules in Behçet's disease

BACKGROUND: Behçet's disease (BD) may lead to blindness in up to 25% of eyes. Soluble (s)ICAM-1 but not sVCAM-1 is associated with relapse in idiopathic uveoretinitis and is reported to be raised in BD patients. We have investigated the levels of sICAM-1 and sVCAM-1 in Palestinian patients with BD and related them to both ocular and systemic disease activity and to immunosuppressive treatment. METHODS: A total of 51 patients (43 male, 8 female; mean ages 29.8 & 31.9 yr) were examined at the St John Ophthalmic Hospital during a one year period (135 consultations). Disease activity was determined from history and standard ocular examination. Anterior uveitis, vitritis and retinal vasculitis acted as markers of ocular inflammation. Peripheral venous sICAM-1 and sVCAM-1 levels were determined by standard ELISA. A total of 53 healthy age- and sex-matched clinic staff members acted as controls. RESULTS: sICAM-1 and sVCAM-1 were both significantly lower in patients on systemic immunosuppression than in those off treatment (p<0.001). Among patients off systemic treatment, sICAM was higher in the group with active systemic disease but quiet eyes (p=0.003), but not in those with active ocular disease (p=0.09), compared to controls. sVCAM was not raised in either group. CONCLUSION: Systemic immunosuppression was associated with reduced sICAM-1 and sVCAM-1, supporting a role for adhesion molecules in the pathogenesis of BD. sICAM-1 levels were raised in association with inflammatory features implicating endothelial activation in active BD. The mean sICAM-1 was higher in active ocular patients than controls, but did not reach statistical significance, emphasising the need for larger studies which include patients with active features of disease but who are off systemic treatment.     

Serum prolactin levels in Behçet's disease

PURPOSE: Evaluation of serum prolactin levels in Beh?et's disease patients in Turkey. METHODS: Serum prolactin levels were measured by radioimmunoassay (RIA) in 17 patients with ocular findings, and 20 patients without ocular findings of Beh?et's disease, and in 17 healthy volunteers. RESULTS: The average prolactin levels were measured as 9.53 ng/mL in patients with ocular findings, 8.84 ng/mL in patients without ocular findings, and 9.59 ng/mL in healthy controls. There was no statistical significance among these three groups. Also, the average levels were 9.84 ng/mL in remission periods and 7.54 ng/mL in attacks. CONCLUSIONS: In some studies, it has been suggested there is a correlation between high serum prolactin levels and activation of some autoimmune diseases, such as systemic lupus erythematosus and rheumatoid arthritis. However, we found no such correlation in Beh?et's disease. On the contrary, prolactin levels were lower in attacks than in remissions.     

Vitreoretinal surgery in Behçet's disease with severe ocular complications

PURPOSE: To investigate effects of vitreoretinal surgery in Beh?et's disease. MATERIALS AND METHOD: Vitreoretinal surgery was applied to 26 eyes of 21 patients with Beh?et's disease. Preoperative and postoperative visual acuities, number and duration of attacks, anterior and posterior segment pathologies were evaluated. RESULTS: The mean age of the patients was 33 years and female/male ratio was 6/15. The mean follow-up was 23 months. Visual acuity increased in 15 eyes (58%), did not change in 11 eyes (42%). In the postoperative period, there was a significant decrease in mean number of uveitis attacks compared to the preoperative period (p=0.001), as well as a significant decrease in the mean duration of uveitis attacks (p=0.001). In the postoperative follow-up, intravitreal haemorrhage in 2 eyes (8%), posterior subcapsular cataract in 5 eyes (19%) and corticonuclear cataract in 2 eyes (8%) were observed. Posterior capsular opacification (PCO) developed in 5 of 16 eyes (31%) having ECLE-IOL. CME continued in 3 eyes (12%). CONCLUSION: Vitreoretinal surgery has favourable effect on the visual and anatomic prognosis in Beh?et's patients with severe ocular complications.     

Clinical study on patients with Behçet's disease in Hokkaido

Beh?et's disease is the most frequently encountered form of endogenous uveitis in Japan. The authors examined the clinical manifestations in cases with Beh?et's disease over six years (1987-1992), and compared the results with those of a previous study (1978-1983). In the more recent study the number of new patients who visited our clinic was 54, compared to 180 in the previous study. As for the frequencies of the four major symptoms in the recent study, oral aphtha were seen in 100%, skin lesions in 88.9%, ocular lesions in 74.0%, and genital ulcers in 61.1% of the patients. The frequencies of these major symptoms were not significantly different between the two time periods investigated. Analysis of the type of ocular involvement showed that 94.4% of the patients in the more recent study belonged to the uveoretinitis type and 5.6% of the patients belonged to the iridocyclitis type, whereas in the previous study the figures were 71.5% and 28.5%, respectively. The ratio of the patients with severe eye involvement has increased recently. However the visual prognosis of patients was better in the more recent study than in the previous study. In the previous study a marked decrease in visual acuity was observed during a two-year follow-up, whereas in the 1987-1992 period it was observed that visual acuity hardly changed over two years. The observed improvement in the visual prognosis may be due to the fact that the treatment of Beh?et's disease has become more effective in recent years.     

Microsatellite polymorphisms of the MICA gene among Japanese patients with Behçet's disease

BACKGROUND: Beh?et's disease (BD) is a multisystemic inflammatory disease of unknown origin. Because some researchers have recently suggested a primary association of BD with the A6 allele of the human major histocompatibility complex class I chain-related A (MICA) gene, we investigated microsatellite polymorphisms of the MICA gene in subjects with and without BD. METHODS: This was a case-control study of 23 Japanese patients with BD and 23 Japanese volunteers without BD who were compared for MICA microsatellite polymorphisms using the polymerase chain reaction (PCR). We also analysed associations between 5 MICA alleles and the clinical features of patients. RESULTS: There was no significant difference between case patients and control subjects in phenotype frequencies. The MICA-A6 allele showed the strongest positive correlation with the human leukocyte antigen allele HLA-B51. Allele A5 showed a strong positive correlation with age at onset and a strong negative correlation with iridocyclitis and HLA-B51. A4 showed a strong negative correlation with ocular lesions and HLA-B51. Patients with the MICA-A6 allele had significantly higher HLA positivity than patients without the allele. INTERPRETATION: While the MICA-A6 allele had no significant association with BD, it showed a strong association with HLA-B51. This finding suggests that an association between MICA-A6 and BD may be a secondary phenomenon related to HLA-B51. As several associations with MICA alleles and clinical features have been found, further investigation is expected to elucidate the biological mechanism of action of the MICA protein relative to disease onset.     

Characteristics of the distribution of major histocompatibility system antigens in retinal vasculitis in patients with Behçet's disease

The etiology of Behcet's disease (BD) has not been accurately specified until now, however, it is a fact of general knowledge that the genetic factors and environment have an effect on the pathogenesis of the disease. 21 histological antigens (HLA) were typed for 30 Caucasians with BD (14 of them had retinal vasculities, and 16 of them were without it). HLA A and HLA B were determined by using the standard lymphocyte microcytotoxicity technique. The disease was diagnosed in accordance with the criteria of the International Study Group for BD. 1000 healthy donors of the Middle European population were in the control group. The frequency of occurrence of HLA B-5 was reliably higher in patients with retinal vasculitis (85.7%, PR = 5.53) and without retinal vasculitis (68.8%, PR = 4.44) as compared to the controls (15.5%, p < 0.0000). The occlusive retinal vasculitis was found only in BD patients with HLA B-5 (p = 0.0242). The study shows that HLA B-5 is associated with BD and can be a marker of severe retinal vasculitis.