Clinical Outcomes of Patients with Vogt–Koyanagi–Harada Disease Over 12 Years at a Tertiary Center

Purpose: To describe the clinical characteristics and treatment outcomes of Vogt–Koyanagi–Harada (VKH) disease over a 12-year period.

Methods: A retrospective chart review was used to identify VKH patients, from January 1999 to December 2011.

Results: In total, 154 patients (308 eyes) were diagnosed with VKH. The mean age at diagnosis was 33.8?±?13 years. Mean baseline best-corrected visual acuity (BCVA) was 20/125; (0.8?±?0.6 logMAR). Recurrent episodes occurred in 107 (54%) patients. Oral prednisone was administered in all patients. At the last visit, the mean BCVA was 20/50 (0.4?±?0.7 logMAR). The most common complications post-treatment were: glaucoma [104 (33.8%) eyes] and cataract [84 (27.2%) eyes].

Conclusions: In Saudi Arabia, VKH-related uveitis is predominant in young females. Bilateral panuveitis is the most common ocular manifestation and near 50% of eyes present with exudative retinal detachment. Oral prednisone was the primary treatment and the majority of eyes maintained 20/50 or better vision.     

A long-term follow-up of Eales’ disease

Purpose: To provide long-term follow-up information on Eales’ patients. Methods: Eales’ patients, who had been examined at varying periods between the years 1970 and 1991 with a minimum five-year follow-up, were included in the study. Results: A total of 130 patients were followed up for a minimum of five and a maximum of 26.5 years. The retinal lesions found during the first examination included vascular sheathing, disc and/or retinal neovascularization, vitreous hemorrhage, branch retinal vein occlusion, retinitis proliferans, and retinal detachment. Visual acuity improved in 37 (20%) of the 185 treated eyes, was maintained in 79 (43%), and worsened in 69 (37%). The complications were tractional detachment, cataract, rubeosis iridis, neovascular glaucoma, and phthisis bulbi. Conclusion: The most important elements in dealing with Eales’ disease are periodic follow-up, a good and adequate laser treatment, pars plana vitrectomy combined with procedures for nonclearing vitreous hemorrhage, and retinal vasoproliferative changes to stabilize the retinal lesions and maintain functional levels of vision.     

A Case of Non-Hodgkin’s Lymphoma Following Long-Term Corticosteroid Therapy for Addison’s Disease

A 76-year-old Japanese woman had suffered from fatigue, weight loss, and cutaneous hyperpigmentation at the age of 38 years and was diagnosed as having tuberculous Addison’s disease. Since then, corticosteroids had been administered effectively as hormonal replacement. At the age of 75 years, the patient presented with a progressive, painless swelling in the left eyelid due to an ill-defined tumor of rubbery consistency in the superotemporal aspect of the orbit. Computed tomography, magnetic resonance imaging, and scintigraphy revealed a wide distribution of tumors, but not in the adrenal gland, which led to the suspicion of systemic malignant lymphoma. Histopathologic examination of the excised orbital tumor was compatible with non-Hodgkin’s lymphoma of the B-cell type. We believe this is the first report of Addison’s disease presenting with non-Hodgkin’s lymphoma. This disease process was characterized by the development of a lymphoid malignancy after long-term corticosteroid therapy to control the adrenal insufficiency, and by the widespread involvement of the lymph nodes and orbit but not the adrenal gland. Corticosteroid-induced abnormal immune state was considered to be the pathogenesis of this unusual complication.     

Long-term Change of Subfoveal Choroidal Thickness in Behçet’s Disease Patients with Posterior Uveitis

Purpose: To evaluate long-term changes of subfoveal choroidal thickness (SCT) in Behçet’s disease (BD) patients with posterior uveitis.

Methods: Changes in SCT measured with enhanced depth imaging optical coherence tomography during quiescent phase were assessed during >24 months in 63 BD patients and control group.

Results: Baseline characteristics showed no difference, but the BD group showed poorer visual acuity (p = 0.013) and smaller SCT (p = 0.006) at final examination. Mean SCT in the BD group decreased from 291.0 to 268.1 μm (p<0.001) during the mean period of 38.5 months. Mean change rate of SCT in the BD group was greater than controls (–7.2 vs 2.0 μm/year; p<0.001) and was associated with longer active inflammation (p<0.001). Patients with longer disease duration showed smaller baseline SCT (p = 0.03).

Conclusions: In BD patients, choroidal thickness decreased over time, which was associated with length of active inflammation. It suggests intraocular inflammation in BD affects the choroid as well as the retina.     

Long-Term Efficacy and Safety of Infliximab in the Treatment of Behçet’s Disease

Purpose: To assess the long-term efficacy and safety of infliximab therapy for the treatment of Behçet’s disease patients with ocular involvement who failed to respond or did not tolerate conventional treatment.

Methods: Retrospective study of 12 patients treated with infliximab at a starting dose of 5?mg/kg.

Results: Infliximab was infused during a mean of 31.43 months. The mean follow-up period was 35.77 months (range: 6?94). All patients achieved remission, 7 of whom did not need any adjuvant immunosuppressive therapy and 9 of whom were able to discontinue systemic corticosteroids. Visual acuity remained stable or improved in 20/21 eyes. Ten patients did not report any side effect of the medication or those were mild and tolerable. We observed two major adverse events requiring withdrawal of infliximab.

Conclusions: Infliximab therapy is an effective biologic agent for the treatment of ocular inflammation in Behçet’s disease unresponsive to the standard immunosuppressive therapy.     

Recurrent Orbital Myositis with Radiological Feature Mimicking Thyroid Eye Disease in a Patient with Crohn’s Disease

Orbital myositis is an extremely rare extra-intestinal manifestation of Crohn’s disease. Herein we describe a patient with self-limiting episodes of episcleritis and uveitis prior to the diagnosis of Crohn’s disease, and subsequent development of recurrent orbital myositis with radiological features mimicking thyroid eye disease.     

Safety of Interferon Alpha-2a in Patients with Severe Ophthalmic Behçet’s Disease: Response to Bielefeld et al.’s Letter

Thyroid dysfunction is a common and severe side-effect encountered in up to 40% of patients treated with IFN-alpha-2a. The main two mechanisms by which IFN-alpha-2a induces thyroid dysfunction can be categorized as autoimmune and non-autoimmune disease. In the first subgroup, thyroid antibodies are found before treatment initiation, and then patients develop thyroiditis. In the second subgroup, IFN-alpha-2a induces thyroiditis by a direct cytotoxic effect on the thyroid gland; in this case, thyroid antibodies are usually negative. To avoid such complications, patients should undergo routine thyroid screening (thyroid-stimulating hormone and thyroid antibodies) prior to IFN-alpha-2a initiation, during the treatment period, and 6 months after treatment withdrawal.     

Role of intravitreal bevacizumab in the management of Eales’ disease

Purpose To evaluate the role of intravitreal bevacizumab in the treatment of Eales’ disease. Study design Retrospective, interventional case series. Methods Two patients with proliferative Eales’ disease were managed with an intravitreal injection of bevacizumab (1.25 mg). The presence of persistent retinal neovascularization despite adequate laser photocoagulation caused recurrent episodes of vitreous hemorrhage in the first patient. The second patient had florid retinal and disc neovascularization with a dispersed vitreous bleed, which prevented laser therapy. Both patients were followed up for 6 months. Results Rapid regression of the retinal neovascularization and clearing of the vitreous hemorrhage were observed in both cases following intravitreal bevacizumab therapy, allowing laser photocoagulation. In both patients, the visual acuity improved, and no signs of recurrence were observed 6 months post-treatment. Conclusion Intravitreal bevacizumab injections may be effective as an adjunctive or alternative treatment of retinal neovascularization in Eales’ disease, where inadequate visualization precludes laser photocoagulation. This approach may also be useful in avoiding vitreoretinal surgical intervention in certain cases. Intravitreal bevacizumab was well tolerated by our patients, and no adverse effects were observed.     

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

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, RD_3, SD_3, 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.     

An Analysis of 1 001 Blinding Patients with Corneal Disease in 1960—1989

Purpose:To investigate the condition and change of corneal blindness (CB) in the past 30 years.Methods: 1 001 blinding patients of corneal disease were clinically analysed from 1960 to 1989,including etiology, sex, age, occupation, difference between the urban and rural areas etc.Results: The male predominated. Infection stood the first cause of blinding corneal diseases, followed by trauma,malnutrition and the others.HSV-1 keratitis was the most frequent infection in 1980s. The highest incidence of CB was 20-40 year -old. The number of CB caused by occupational truauma was more than that by ordinary trauma in 1960s,but from 1970s, the latter was more than the former. The number of CB in rural area was more than that in the urban in 1960s,but from 1970s,there was no significant difference between the two areas. The percentage of bilateral CB was gradually decreased.Conclusion: We should try to find more effective strategies to prevent and treat CB caused by HSV-1 keratitis, pay special attention to pres     

Designing Manufacturing and the Clinic Application of the Prosthesis for the Patients with Large Ocular Defects

Purpose: To design and manufacture the prosthesis of external type for patients with ocular large defects.Methods: Silicon and polymethyl methacrylate ( PMMA) were chosen the chief materials: The main procedures are: taking the impression, color mixture, making primitive form of the prosthesis, modification and fixation.Results: Fifty patients with large ocular defects took the prosthesis, the cosmetic results are satisfactory. All the patients give satisfactory comments on the 5 main indexes; color 90% , stereofeeling 100% , peripheral difference 86% , fidelity 98% and fixation 86% . Conclusions: To make ocular prosthesis, materials with good quality and complicated technology are needed. The prosthesis made by silicon combined with PMMA show good results. Among the procedures of making prosthesis, color mixture and fixation are the most important . Eye Science 1997; 13:141 - 143.     

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

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.     

Ocular Blood Flow Changes in Behçet Disease Patients with/without Thrombotic Disease

ABSTRACT

In this study, the authors aimed to evaluate ocular blood flow changes in Behçet disease (BD) with and without thrombotic disease. Ninety eyes of 90 patients with a diagnosis of BD (30 eyes with active uveitis, 23 eyes with inactive uveitis, 25 eyes without ocular involvement, and 12 eyes without ocular involvement and with a history of thrombosis) and 30 eyes of 30 age- and sex-matched control patients without any systemic disease with a total of 120 eyes were evaluated. In all cases, ophthalmic, central retinal, and ciliary artery flow parameters were measured with colour Doppler ultrasonography (CDU). The ocular blood flow parameters of all vessels in patients with active uveitis were found to be affected. All the flow parameters in the CRAs of the study groups were significantly different from the control group (p?<?0.001). Additionally, in non-ocular BD patients with thrombosis, blood flow parameters were affected more than the parameters in non-ocular BD patients without thrombosis and control patients. In conclusion, major haemodynamic changes were observed using CDU in the ophthalmic vessels of ocular Behçet patients. Also, CDU may detect ocular blood flow alterations before initial ocular clinical manifestations appear in BD patients     

Graves’ Disease Associated with Primary Systemic Sclerosis

Graves’ disease can be associated with other autoimmune disorders. Primary systemic sclerosis (PSS) is such a disease characterised by multi-organ fibrosis. Abnormal thyroid antibody titres and hypothyroidism as well as hyperthyroidism have been observed in PSS patients. Specific and idiopathic inflammatory disorders are an important differential diagnosis in Graves’orbitopathy (GO). Mycophenolate mofetil (MM) is an immunosuppressive drug which depletes guanosin nucleotides in proliferating B-and T-lymphocytes. We describe the effect of MM on the endocrine orbitopathy of a patient with PSS.     

Adalimumab Treatment in Patients with Vogt–Koyanagi–Harada Disease

Purpose: To evaluate the clinical outcome and safety of adalimumab in patients with Vogt–Koyanagi–Harada (VKH) disease.

Methods: VKH patients treated with adalimumab seen at the University of Buenos Aires were reviewed. Main outcome measures were visual acuity, anterior segment inflammation, optic nerve inflammation (ONI), steroid sparing effect, number of immunosuppressives, and relapses.

Results: In total, 14 VKH patients, mean age 23.07 ± 8 years; median of adalimumab treatment 10 months, were analyzed. At start of adalimumab treatment (baseline), median of corticosteroid dose was 20 mg and at 6 months, 4 mg. At baseline, 11 patients were on immunosuppressive treatment and at 6 months only four continued with immunosuppressive therapy. In the 28 eyes, the median of active inflammation was 2 at baseline and 0 after 6 months on adalimumab.

Conclusions: Treatment with adalimumab is an effective and safe option, reducing the need for oral corticosteroid and conventional immunosuppressive therapy.     

A Case of Coats’ Disease with Spontaneous Retinal Reattachment after Total Detachment

PurposeTo report a case of Coats’ disease in which spontaneous reattachment occurred after total retinal detachment.ResultsFour months after the initial diagnosis, the retina showed complete reattachment with a large amount of subretinal hard exudate. Visual acuity remained unchanged, with no light perception.ConclusionsWe speculate that the spontaneous retinal reattachment in the present case was caused by the decreased permeability of the abnormal retinal vessels and the good functional effect of the retinal pigment epithelium.Key Words: Coats’ disease, Retinal detachment, Spontaneous reattachment of the retina, Subretinal exudate     

Evaluation of a Relative Afferent Pupillary Defect using the RAPDx® Device Before and After Treatment in Patients with Optic Nerve Disease

We evaluated the amplitude and latency scores in the RAPDx® device together with other ophthalmic examinations, before and after treatment in four patients with optic nerve disease. In all patients, the visual acuity (VA) and visual field (VF) after treatment was resolved. Both scores after treatment were lower, with reduced laterality-based differences in VA and critical flicker fusion frequency (CFF). Even after treatment, 3 patients had laterality-based differences in circumpapillary retinal nerve fiber layer thickness (cpRFNLT). Both scores for evaluation of RAPD by RAPDx® correlated with subjective examinations and were useful for evaluation of the efficacy of treatment.