Didar Ucar

Canakinumab in a patient with juvenile Behçet's syndrome with refractory eye disease

Behcets syndrome (BS) causes panuveitis and retinal vasculitis in about 50% of patients. Despite intensive treatment, up to 20% of patients may lose useful vision.1 Treatment consists of corticosteroids, immunosuppressive agents such as azathioprine (AZA) and ciclosporin A (CycA),1 and biological agents such as interferon-α (IFN) and antitumour necrosis factor agents.2 ,3 We describe a patient with juvenile BS whose refractory eye disease was treated successfully with canakinumab, a fully human anti-interleukin-1β antibody. Figure 1 shows the different treatment regimes used during the follow-up period. Figure 1 Different treatment regimes and eye flares during the follow-up period. A 16-year-old girl was diagnosed with BS at the age of 9 years because …

Glutathione S transferase M1 and T1 genetic polymorphisms are related to the risk of primary open-angle glaucoma: a study in a Turkish population.

Background: Genetic factors and oxidative damage have been shown to have a role in the development of primary open angle glaucoma (POAG). Aim: To determine the effects of genetic polymorphisms of glutathione S transferase (GST)M1 and GSTT1 on the risk of POAG in a Turkish population. Methods: Using a multiplex polymerase chain reaction (PCR), GSTM1 and GSTT1 gene polymorphisms were analysed in 144 patients with POAG and in 121 otherwise healthy controls of similar age. Results: The GSTM1 positive genotype and the GSTT1 null genotype had an increased risk of developing POAG (p<0.001, OR 2.93, 95% CI 1.66 to 5.20 and OR 4.25, 95% CI 2.30 to 7.80, respectively). The risk of glaucoma also increased significantly in subjects with a combination of GSTM1 positive and GSTT1 null genotypes (p<0.001, OR 3.46, 95% CI 1.64 to 7.38). Conclusion: The GSTM1 positive genotype and GSTT1 null genotype or the combination of both may be associated with the increased risk of development of POAG in the Turkish population.

Scleral Buckling with a Non-Contact Wide-Angle Viewing System

Purpose: To assess the outcome of scleral buckling surgery using a non-contact wide-angle viewing system for fundus visualization in patients with rhegmatogenous retinal detachment without proliferative vitreoretinopathy. Patients and Methods: Sixteen eyes of 16 patients underwent scleral buckling using a non-contact wide-angle viewing system combined with a 25-gauge illumination fibre inserted into the sclera at the pars plana. Results: The mean age of the patients was 53.6 ± 13.7 years and the mean follow-up time was 13.4 ± 2.8 months. Retinal reattachment was achieved in 13 of the 16 eyes (81%). Three eyes underwent vitrectomy with silicone oil injection because of development of proliferative vitreoretinopathy in 2 eyes and scleral perforation due to excessive indentation during cryoretinopexy in 1 eye. Two eyes developed limited subretinal haemorrhage during subretinal fluid drainage. Conclusion: Simultaneous use of a non-contact wide-angle viewing system combined with a 25-gauge light fibre illumination for fundus visualization brings the advantages of microsurgery and indirect ophthalmoscopy into scleral buckling surgery.

Ocular Involvement of Behçet’s Syndrome: a Comprehensive Review

Behçet’s syndrome (BS) is a vasculitis involving several organ systems including the eyes. Ocular involvement is one of the most disabling complications of BS, causing loss of vision that may progress to blindness if left untreated. The typical form of ocular involvement is a relapsing and remitting panuveitis and retinal vasculitis. Initial attacks may spontaneously improve and subsequently disappear in a few weeks but tend to recur if left untreated. Destructive and recurrent attacks, especially with posterior segment and retina involvement, may cause irreversible ocular structural changes and permanent damage in sensory retina, resulting in loss of vision. The risk of irreversible damage to ocular tissue which may result in loss of vision warrants early and intensive treatment especially in patients at high risk such as young men who tend to follow an aggressive disease course. The management strategy involves rapid suppression of inflammation during the attacks and prevention of recurrent attacks. Local and systemic measures including immunosuppressives, corticosteroids, and biologic agents are used for this purpose. Surgery may be required in selected cases. The prognosis of eye involvement has greatly improved over the last decades with the effective use of immunosuppressives.

Behçet’s syndrome: providing integrated care

Behçet’s syndrome (BS) is a multisystem vasculitis that presents with a variety of mucocutaneous manifestations such as oral and genital ulcers, papulopustular lesions and erythema nodosum as well as ocular, vascular, gastrointestinal and nervous system involvement. Although it occurs worldwide, it is especially prevalent in the Far East and around the Mediterranean Sea. Male gender and younger age at disease onset are associated with a more severe disease course. The management of BS depends on the severity of symptoms. If untreated, morbidity and mortality are considerably high in patients with major organ involvement. Multidisciplinary patient care is essential for the management of BS, as it is for other multisystem diseases. Rheumatologists, dermatologists, ophthalmologists, neurologists, cardiovascular surgeons and gastroenterologists are members of the multidisciplinary team. In this study, we reviewed the epidemiology, etiology, diagnostic criteria sets, clinical findings and treatment of BS and highlighted the importance of the multidisciplinary team in the management of BS.

An Exceptional Case of Full-thickness Macular Hole Closure in a Patient with Behçet Disease

Abstract Purpose: To present a case of a full-thickness macular hole closure in a patient with Behcet uveitis Design: A 23-year-old-male patient with Behcet Disease had clinically inactive uveitis with topical steroids, oral azathiopurine and cyclosporine until he developed a retinal infiltrate in the left eye which evolved into a full-thickness macular hole during the follow-up. Methods: Strict control of inflammation and subsequent vitrectomy was planned. Meanwhile another attack of panuveitis developed in the left eye and subcutaneous Interferon alfa-2b interferon treatment was initiated. Results: After 2 months, the patient was clinically inactive with complete closure of the macular hole. Conclusions: Strict control of inflammation may result in closure of the macular hole and avoid the need for vitrectomy.

Optical Coherence Tomographic Findings in Susac Syndrome

Purpose: To report optical coherence tomographic (OCT) findings in Susac syndrome. Methods: A 15-year-old girl who had symptoms of deafness and encephalopathy was referred with a provisional diagnosis of Susac syndrome. Funduscopy revealed cotton wool spots and ischaemic cloudy swelling of the retina. Results: OCT taken from the abnormal retinal area showed increased reflectivity of the inner retinal layers and decreased reflectivity from the outer retinal layers and the retinal pigment epithelium due to shadowing. Neither intraretinal nor subretinal fluid were observed on the OCT imaging. OCT findings were compatible with the accepted view that the retinal pathology in Susac syndrome is due to arteriolar occlusion. Conclusion: OCT may be used as a complementary diagnostic method to fluorescein angiography in Susac syndrome.

Histologic Findings of Corneal Buttons in Decompensated Corneas With Toxic Anterior Segment Syndrome After Cataract Surgery.

Purpose: To report the histologic findings of penetrating keratoplasty buttons in decompensated corneas with toxic anterior segment syndrome (TASS) after a cataract surgery is performed. Methods: We evaluated the histologic findings of 16 corneal buttons of 16 patients who exhibited decompensation findings because of TASS by means of light microscopy. The patients were classified into 3 groups: The first group consisted of 5 corneal buttons with mild symptoms in which the central corneal thicknesses (CCTs) were ⩽650 &mgr;m, and the visual acuities (VAs) were ≥0.1. The second group consisted of 7 corneal buttons that exhibited moderate symptoms in which the CCTs were between 650 and 750 &mgr;m and the VAs were between 0.1 and 0.03. The third group consisted of 4 corneal buttons that had severe symptoms in which the CCTs were ≥750 &mgr;m and the VAs were ⩽0.03. Results: Light microscopy showed endothelial cell loss, vacuolated and thinned epithelial cell layers, disturbed collagen bonds, and Descemet membrane invaginations in patients in group I. Group II corneal buttons exhibited inflammatory cells (lymphocytes) and extended intercellular space between the epithelial cells, wrinkled Bowman membrane separated from the stroma in some local areas, stromal edema, and early vascularization. In group III, endothelial and epithelial cell layer loss, wrinkled Descemet and Bowman membranes, inflammatory cells, and structurally disturbed collagen bonds located beneath the Bowman membrane, and a greater amount of vascularization in the area of inflammation were observed. Conclusions: The results of the histologic evaluation of the decompensated corneas caused by the TASS are compatible with the clinical severity of the disease. In mild cases, the histologic findings were insignificant; however, when the clinical situation deteriorated, histologic findings became increasingly worse.

Retinal Nerve Fiber Layer Thickness Alterations after Electroconvulsive Therapy in Patients with Mental Illness

ABSTRACT Background: We hypothesized that the ongoing neurotrophic effects of electroconvulsive therapy (ECT) might be detected in the retinal nerve fiber layer (RNFL) using optical coherence tomography (OCT). Methods: This study was conducted using a prospective design. The RNFL thickness alterations of 18 psychiatric patients undergoing ECT were assessed using OCT. The results were compared with the RNFL thicknesses of 18 age and gender-matched healthy controls. Results: In the left eye, the average RNFL (p = 0.025), superior quadrant (p = 0.029), and nasal quadrant (p = 0.021) thicknesses were significantly increased after the last ECT treatment. In contrast to the right eye, the basal left-eye superior RNFL thickness (p = 0.038) and inferior quadrant (p = 0.007) thicknesses were significantly lower in the patients than in the healthy controls. Conclusion: Here, we have revealed a significant lateralized influence of ECT on the left-eye RNFL. However, larger case series should be conducted before interpreting our current findings.

Temperament and Character Traits in Patients with Behçet’s Disease with/without Eye Involvement

ABSTRACT Purpose: Ophthalmic involvement may lead to permanent vision loss in 25% of cases in patients with BD and it is a main concern in the literature. Although several studies have been investigated, the etiology and the cause of the disease and attacks are not yet known. This study aimed to investigate the correlation between visual impairment and personal characteristics and social circumstances in patients with BD. Materials and Methods: A total of 153 patients with BD and age-and gender-matched 26 healthy control subjects completed the self-report Temperament and Character Inventory (TCI), Beck Depression Inventory (BDI), and State and Trait Anxiety Inventory (STAI-S and STAI-T). We classified the study participants into three groups with respect to severity of eye involvement and one control group. Each group was compared with the other two study groups and control group. Results: According to TCI, we revealed that there was a trend in BD patients with eye involvement + poor prognosis having less disorderliness traits than BD patients with eye involvement + good prognosis (p = 0.016). The BD patients with eye involvement + poor prognosis had significantly lower attachment scores than BD patients with eye involvement + good prognosis (p = 0.005) and healthy controls (p = 0.005). The BD with eye involvement + poor prognosis had lower empathy scores than healthy controls (p = 0.002). In the way of average TCI parameters, only SD was statistically significant. In terms of subdimensions of TCI parameters, RD3, SD3, SD5, and C2 were shown to be statistically significant among some of the groups. Conclusion: BD patients with eye involvement were demonstrated to be more extravagant and socially disinterested. It may reflect that severe visual loss caused BD patients to be more systematic, depressive, self-contained, and exhausted. Considering psychological aspects of BD and its visual manifestations may contribute to helping these patients more effectively.