Sibel Kadayifcilar

Is elevated level of soluble endothelial protein C receptor a new risk factor for retinal vein occlusion

Background:  To evaluate the systemic and thrombophilic risk factors for retinal vein occlusion (RVO) and to determine whether the elevated level of soluble endothelial protein C receptor (sEPCR) is a risk factor for thrombosis.

Bilateral acute iris transillumination

OBJECTIVE To describe a series of patients with bilateral acute iris transillumination, pigment dispersion, and sphincter paralysis. METHODS We reviewed the medical records and clinical photographs of 26 patients seen at 5 centers in Turkey and Belgium between March 16, 2006, and July 6, 2010. Observation procedures included clinical examination, anterior segment color photography, gonioscopy, laser flare photometry, and pupillometry. RESULTS All 26 patients (20 women and 6 men; mean [SD] age, 43.2 [10.5] years) had bilateral involvement. Twenty-three patients (88%) had acute-onset disease with severe photophobia and red eyes. Nineteen patients (73%) had a preceding flulike illness and used systemic antibiotics, including moxifloxacin. Diagnostic laboratory workup was unremarkable. There was pigment discharge into the anterior chamber, and flare was elevated in the absence of inflammatory cells. Most patients had severe diffuse transillumination of the iris and mydriatic distorted pupils. Pupillometry revealed a compromised reaction to light. The most serious complication was an intractable early rise in intraocular pressure. Gonioscopy revealed heavy pigment deposition in the trabecular meshwork. Although symptoms were relieved promptly by application of topical corticosteroid, the median duration of pigment dispersion was 5.25 months. CONCLUSIONS Bilateral acute iris transillumination with pigment dispersion and persistent mydriasis is a new clinical entity that is not an ocular adverse effect of oral moxifloxacin treatment, as previously suggested. The etiopathogenesis of this entity remains to be elucidated.

Cataract surgery in patients with Behçet’s disease

Purpose: To evaluate the outcomes and complications of cataract surgery in patients with Behçets disease. Setting: Department of Ophthalmology, Hacettepe University School of Medicine, Ankara, Turkey. Methods: Thirty‐three eyes of 26 patients with Behçets 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 Behçets 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 Behçets 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.

Safety and Efficacy of Gevokizumab in Patients with Behçet’s Disease Uveitis: Results of an Exploratory Phase 2 Study

ABSTRACT Purpose: To evaluate the safety and efficacy of gevokizumab for the treatment of Behçet’s disease uveitis in a prospective, open-label, randomized phase 2 trial. Methods: Behçet’s disease patients with new acute ocular exacerbation or at risk of exacerbation received 30 or 60 mg gevokizumab every 4 weeks intravenously or subcutaneously, on top of a stable regimen of immunosuppressives and corticosteroids (≤20 mg/day equivalent prednisolone). Patients withdrew in cases of ocular exacerbation. Results: A total of 21 patients were included (17 acute and 4 at-risk; mean duration of uveitis 45.6 ± 37.4 months). There were no serious adverse events related to gevokizumab. Recorded adverse events were mostly associated with exacerbation of uveitis or its complications. Response was evaluated for 14 acute patients and all showed rapid control of acute ocular exacerbation, mostly within 1 week, without any increase in corticosteroid dosage. Conclusions: Gevokizumab was well tolerated and rapidly controlled acute ocular exacerbations of Behçet’s disease uveitis without the need for high-dose corticosteroid.

Keratic precipitate morphology in uveitic syndromes including Behçet's disease as evaluated with in vivo confocal microscopy.

PurposeTo identify the morphologic appearance of keratic precipitates (KPs) with in vivo confocal microscopy (IVCM) in uveitic syndromes.MethodsA total of 75 eyes of 72 patients with a mean age of 38.6±15.1 years who had active intraocular inflammation and whose corneas had KP on slit-lamp examination were included in this study. IVCM (Confoscan 3.0, Vigonza, Italy) was used to image the part of the corneal endothelium in which KP were most densely deposited. KP were classified into five groups: type I (small, round), type II (stippled), type III (dendritiform), type IV (large, smooth-rounded), and type V (globular). When more than one type of KP was observed with IVCM, a distinction between the predominant and the less frequent KP was made as ‘primary’ and ‘secondary’ KP.ResultsIn 50 (66.7%) eyes more than one type of KP was imaged. The size of the KP ranged between 5 and 150 μm. The most frequently observed primary KP type in Behçets disease was type I (100%), in ankylosing spondylitis type II (57.1%), in Fuchs’ uveitis syndrome type III (85.7%), in granulomatous uveitis type V (42.9%), in infectious uveitis type III (66.7%), and in juvenile idiopathic arthritis associated uveitis type I (66.7%). The KP types showed a statistically significant difference between different uveitic syndromes (Fishers exact test, P<0.001).ConclusionsCertain KP types appear to be characteristic of various uveitic syndromes. IVCM may have a potential role in the diagnostic work-up of uveitic patients.

In vivo confocal microscopic evaluation of keratic precipitates and endothelial morphology in Fuchs' uveitis syndrome

PurposeTo evaluate the endothelial cell layer in patients with Fuchs’ uveitis syndrome (FUS) with respect to the type and distribution of keratic precipitates (KP), endothelial cell morphology, and endothelial cell density (ECD), using in vivoconfocal microscopy (IVCM).MethodsForty eyes of 40 patients (mean age of 32.2±12.5 years) with the clinical diagnosis of FUS were evaluated with IVCM (Confoscan 3.0, Vigonza, Italy). KP were classified as type I (small, round), type II (stippled), type III (dendritiform), and type IV (globular). When >1 KP type was present, differentiation between the predominant and less frequent KP was made as ‘primary’ and ‘secondary’. ECD was measured and compared with age-matched 60 control subjects. Endothelial blebs were classified as small (3–10 μm) or large (>10 μm).ResultsIn 36 (90.0%) cases with FUS, more than one KP type was observed with IVCM. Type III (dendritiform) KP was the most frequently observed primary KP type (85.0%), followed by type II (stippled) KP (15.0%). Secondary KP included type II (58.3%), type IV (globular) (27.8%), and type III (13.9%). The mean endothelial cell density of eyes with FUS (2588±396 cells/mm2) was significantly lower than that of control subjects (2930±364 cells/mm2) (t-test; P<0.001). Eyes with FUS had lower proportion of hexagonal cells and higher percentage of polymegethism compared with the uninvolved contralateral eyes. Endothelial blebs (21 small, 16 large blebs) were observed in 37 (92.5%) eyes.ConclusionsFUS is characterized by dendritiform KP and is associated with decreased ECD and altered endothelial cell morphology.

Panuveitis associated with multiple sclerosis complicated by cerebral venous thrombosis.

Purpose: To report cerebral venous thrombosis as a complication of intravenous corticosteroid treatment in a patient with multiple sclerosis. Method: A case report. A 44-year-old woman with a previous diagnosis of multiple sclerosis presented with panuveitis and retinal perivasculitis. Intravenous pulse corticosteroid therapy was given for three days. Results: The panuveitis and retinal periphlebitis began to resolve within one week; however, ten days after the last corticosteroid dose, the patient was hospitalized with the diagnosis of cerebral venous thrombosis. Conclusion: Although intravenous corticosteroid treatment for uveitis associated with multiple sclerosis can be very helpful, the patient should be monitored closely for systemic side effects.

The role of IgM isotype anticardiolipin antibodies in occlusive ocular vascular disease: report of two cases with primary antiphospholipid antibody syndrome.

The role of IgM isotype anticardiolipin antibodies in occlusive ocular vascular disease: Report of two cases with primary antiphospholipid antibody syndrome

Rheumatic Inflammatory Eye Diseases of Childhood

Chronic inflammatory eye diseases are a common manifestation of pediatric rheumatologic diseases, potentially leading to lifelong vision impairment and disability. The mechanisms leading to the breach of the blood eye/brain barrier and the subsequent immune attack against a variety of intraocular mostly unidentified antigens remains poorly understood. Pediatric rheumatologists need to be familiar with the various inflammatory eye diseases because they are often responsible for selecting and supervising treatment in close collaboration with the ophthalmologist. This article provides an update of recent developments in the pathogenesis and treatment of the most relevant ocular diseases encountered in rheumatologic practice.

Sympathetic ophthalmia associated with ocular and cerebral vasculitis: an angiographic and radiologic study.

Purpose To describe a case of sympathetic ophthalmia (SO) associated with ocular and cerebral vasculitis. Methods The authors report a 38-year-old woman who presented with bilateral uveitis 7 years after a penetrating trauma to the right eye. Ocular examination included fundus fluorescein and indocyanine green angiography. Medical history disclosed an episode of dysarthria and right-sided weakness. Results Anterior uveitis was present in the previously injured pseudophakic right eye, which harbored a piece of glass stuck in the lower temporal iris. In the left eye, vitreal inflammation with retinal sheathing and subretinal lesions was predominant. Besides retinal vasculitis, fundus angiography showed choroidal vasculitis. Neurologic studies were compatible with cerebral vasculitis. Conclusions In SO, choroidal vasculitis that is invisible clinically can be demonstrated angiographically. Although rare, inflammation can also affect the nervous system.