Clinical Profile,Treatment, and Visual Outcome of Serpiginous Choroiditis

The purpose of this study was to report the clinical profile and management of patients with serpiginous choroiditis in a tertiary care referral center in India. In a retrospective cohort study, 107 eyes of 70 patients with serpiginous choroiditis seen between January 1995 and December 2002 were analyzed. Systemic steroids and immunosuppressives were the mainstay of therapy. Antituberculous and antiviral drugs were used in selected cases. There was male preponderance (7:3). Age at presentation ranged from 11 years to 52 years (mean 30.3 ± 9 years); 52.9% had bilateral involvement. Vision improved or maintained in 86% eyes and deteriorated in 15 eyes (14%). The main cause of decrease of vision was macular involvement. Improvement in vision and resolution of lesions in patients with serpiginous choroiditis can occur with combination therapy of systemic steroids and immunosuppressive agents. Serial examination at regular intervals is needed to monitor the disease progression, recurrences, and involvement of the other eye.     

Treatment of Serpiginous Choroiditis with Chlorambucil: A Report of 17 Patients

Purpose: To evaluate the efficacy of chlorambucil in the treatment of serpiginous choroiditis.

Methods: Patient records from the Massachusetts Eye Research and Surgery Institution (MERSI) were reviewed from over the past 10 years. In total, 17 patients with the diagnosis of serpiginous choroiditis treated with chlorambucil were identified. QuantiFERON gold was negative in all of them. Chlorambucil was started at 0.15 mg/kg and dosage was titrated up using weekly white blood cell (WBC) count to achieve a target cell number of 3.0–4.5 × 10⁹ cells/L. The goal of therapy was to maintain this value for at least 6–9 months. Adverse effects, recurrence, rate of new choroidal neovascularization (CNVM), and visual acuity before and after treatment were recorded.

Results: The mean age of the 17 patients with the diagnosis of serpiginous choroiditis treated with chlorambucil was 46 years, and six patients (35%) were male. The mean duration of treatment for chlorambucil was 8.4 months. None of them developed cancer or persistent side-effects, with a mean follow-up of 53 months. Of the patients, 12 (71%) achieved an average of 45 (5–120) months drug-free remission in their last follow-up. Visual acuity of 33 treated eyes remained within two lines of Snellen acuity in 27 eyes (82%), improved in one eye (3%), and deteriorated in five eyes (15%). Leukopenia was the most common side-effect, which was reversible in all cases.

Conclusions: Chlorambucil in a relatively short duration of time, with an escalating dose guided by weekly WBC was well tolerated, as well as effective in preventing recurrence and maintaining vision in patients with serpiginous choroiditis.     

Serpiginous choroiditis and acute retinal necrosis occurring in the same patient

We describe a rare association of serpiginous choroiditis with necrotizing retinitis having clinical features of acute retinal necrosis (ARN). A 23-year-old male developed ARN in the fellow eye while he was on tapering doses of immunosuppressive medications for unilateral serpiginous choroiditis. The association may represent a common viral etiology of the two diseases or may be due to the development of ARN due to general state of iatrogenic immunosuppression. This report also highlights the importance of a detailed evaluation of both the eyes on regular follow-up visits in the patients receiving iatrogenic immunosuppression.     

Multimodal Evaluation of Patients with Acute Posterior Multifocal Placoid Pigment Epitheliopathy and Serpiginous Choroiditis

Purpose: To evaluate retinal and choroidal changes in patients with non-granulomatous choroiditis using the multimodality imaging (MMI).

Methods: Eight eyes of four patients were analyzed. Three patients (six eyes) were diagnosed with serpiginous choroiditis (SC) and one patient (two eyes) with acute posterior multifocal placoid pigment epitheliopathy (APMPPE). The patients were imaged on the same day using the RTVue Avanti XR instrument OCT/OCTA (Optovue Inc, Fremont, CA), Heidelberg Retina Angiograph 2 FAF and FA (Heidelberg Engineering, Germany), and TRC50DXi Topcon FP (Topcon Medical Systems, Oakland, NJ).

Results: OCT angiography (OCTA) showed hypoperfusion in all the cases, reperfusion in choriocapillaris in two eyes after treatment and identified a choroidal neovascularization (CNV), which was not detected on the fluorescein angiography (FA).

Conclusions: OCTA may be an effective noninvasive image modality to follow up these patients and may provide further information to help us to understand the pathophysiology and complications of these diseases.     

Combination of azathioprine and corticosteroids in the treatment of serpiginous choroiditis

BACKGROUND: Although therapy with immunosuppressive agents is currently accepted as the best option for treating active serpiginous choroiditis (SC), there is no consensus on the most effective immunosuppressive drug to use. In this paper, we describe the clinical course of patients with active SC treated with azathioprine (AZA) in combination with corticosteroids. METHODS: This retrospective study included 4 patients (5 eyes) with active, vision-threatening SC who received systemic immunosuppression with AZA at 1.5 to 2.0 mg/kg per day. In combination with oral AZA, patients also received 1 mg/kg oral prednisone per day. Information collected included Snellen visual acuity (VA), clinical disease activity, duration of follow-up, rate of inflammation recurrence, and side effects of AZA. RESULTS: Within 3 weeks of treatment, all patients experienced decreased ocular inflammation and improved VA. One patient, however, had a recurrence in both eyes while oral prednisone was being tapered. In this case, once the dosage of oral prednisone was increased and methotrexate was added to the therapeutic scheme, inflammation was controlled within 1 month. The other 3 patients presented no further visual loss while on AZA and were able to taper and then discontinue oral prednisone. Nevertheless, SC recurred in 1 of these patients 40 months after the initial treatment. AZA was reintroduced but the patient complained of gastrointestinal problems, and it was then successfully replaced by mycophenolate mofetil. None of the 4 patients presented serious systemic side effects secondary to AZA. INTERPRETATION: This study suggests that when AZA is used in combination with corticosteroids it is a safe and acceptable option for treating patients with active SC. Side effects and recurrences while on AZA therapy can occur, requiring either replacement of the drug or addition of another immunosuppressive agent.     

Clinical Profile,Treatment, and Visual Outcome of Ampiginous Choroiditis

Purpose: To report the clinical profile and management of patients diagnosed to have ampiginous choroiditis in a tertiary care referral centre in India.

Methods: Retrospective cohort study. Twenty-six eyes of 16 patients were included in the study, which was diagnosed as choroiditis, serpiginous choroiditis, and acute posterior multifocal placoid pigment epitheliopathy (APMPPE) or ampiginous choroiditis. Those who were initially diagnosed as having other forms of choroiditis were later classified as having ampiginous choroiditis clinically. Systemic steroids and immunosuppressives were the mainstay of therapy.

Results: There was a male preponderance (7:3). Age at presentation ranged from 22 years to 57 years with a (median 34 years); 81% had bilateral involvement and 35% had recurrences. Vision improved or maintained in 24 eyes, whereas it deteriorated in 2 eyes due to subretinal fibrosis and macula involvement, respectively. Resolution of lesions and improvement or stability of vision can occur with administration of timely steroids and immunosuppressive therapy. Regular follow-up is necessary to monitor the disease progression, recurrences, and involvement of the other eye.

Conclusion: Ampiginous choroiditis is a separate disease entity due to its distinct clinical features. It is a disease with multiple relapses, which can be effectively controlled with a combination of immunosuppressive therapy, and a good visual acuity can be maintained on long-term follow-up.     

糖皮质激素和环磷酰胺及奥曲肽治疗Graves眼病的临床疗效

目的:探究糖皮质激素、环磷酰胺、奥曲肽三种药物治疗Graves眼病(GO)的临床疗效。方法:回顾性研究。选取2018-06/2019-10我院收治的GO患者102例152眼,按治疗方式分为糖皮质激素组(33例51眼)、环磷酰胺组(38例59眼)与奥曲肽组(31例42眼),总疗程12wk。比较三组患者治疗效果,治疗前后眼球突出度、复视、眼内压、视力变化情况,并进行甲状腺相关眼部活动度评分(CAS);经眼部超声检查眼轴、球横径、球尖距、球后软组织周长、面积及体积的变化;测定治疗前后促甲状腺激素受体抗体(TRAb)、甲状腺过氧化物酶抗体(TPOAb)及甲状腺体积的变化;统计不良反应发生情况。结果:糖皮质激素组、环磷酰胺组患者疗效均优于奥曲肽组(P<0.05),但糖皮质激素组与环磷酰胺组疗效无差异(P>0.05)。治疗12wk,三组患者眼球突出度、CAS评分均降低(P<0.05),视力及自觉复视情况均改善,球尖距、球后软组织周长、面积及体积、TRAb、TPOAb、甲状腺体积均降低(P<0.05),且糖皮质激素组、环磷酰胺组患者眼球突出度、CAS评分、球尖距、球后软组织周长、面积及体积、TRAb、TPOAb、甲状腺体积均低于奥曲肽组(P<0.05),但糖皮质激素组、环磷酰胺组组间以上参数比较均无差异(P>0.05)。治疗期间,糖皮质激素组体质量增加及总不良反应发生率均高于环磷酰胺组与奥曲肽组(P<0.0167),但环磷酰胺组、奥曲肽组组间不良反应发生率无差异(P>0.0167)。结论:糖皮质激素、环磷酰胺治疗GO整体疗效优于奥曲肽,对眼征及甲状腺相关病变改善优于奥曲肽,但环磷酰胺治疗不良反应较糖皮质激素低,安全性更高,可作为GO治疗的首选。     

Thermal Profile of Pulse Precision Capsulotomy: In Vivo and In Vitro Infrared Thermography Study

PurposeThis study aimed to elucidate the thermal safety of precision pulse capsulotomy (PPC) via in vivo and in vitro evaluation of the thermal profile using infrared thermography.MethodsThis prospective observational study enrolled 15 eyes from 15 participants who underwent cataract surgery using the Zepto PPC. All patients underwent temperature measurements of the incision site and the entire cornea using an infrared thermographer during the capsulotomy procedure. To accurately analyze the temperature change of the Zepto PPC, infrared thermography was performed with the Zepto handpiece while exposed to air and then in porcine eyes. Moreover, in each case, the difference in temperature change according to the use of an ophthalmic viscosurgical device (OVD) was also checked to determine the temperature buffering effect.ResultsIn the clinical evaluations, the mean temperature elevation around the corneal incision and time duration from baseline to peak temperature during the Zepto capsulotomy were 4.0°C ± 1.9°C and 4.43 ± 1.26 seconds, respectively, with a mean peak temperature of 32.6°C ± 2.0°C. The mean peak temperature and rise time of the naïve Zepto nitinol ring, as measured from the bottom side, were 109.0°C ± 22.9°C and 43.40 ± 11.06 seconds in the experimental procedures, respectively. In the porcine eyes, the mean elevation of temperature and rise time of the Zepto nitinol ring were 6.2°C ± 1.6°C and 11.67 ± 2.08 seconds with the use of OVDs, and 10.5°C ± 3.3°C and 14.00 ± 3.61 seconds without OVDs, respectively.ConclusionsZepto PPC has the potential to generate extremely high thermal energy, according to an in vitro study. However, the temperature rise of the Zepto capsulotomy can be minimized by using OVDs.     

Macular and Retinal Nerve Fibre Layer Thinning in Xeroderma Pigmentosum: A Cross-sectional Study

The purpose of this study was to evaluate retinal thickness in different Xeroderma Pigmentosum (XP) complementation groups using spectral-domain optical coherence tomography (SD-OCT).

This was a cross-sectional pilot study of 40 patients with XP. All patients had healthy-looking retinae and optic nerves on slit lamp biomicroscopy, and subtle or no neurological deficits. Patients were divided into two groups based on the known tendency for neurodegeneration associated with certain XP complementation groups. A third control group was obtained from a normative database. Using SD-OCT, we compared peripapillary retinal nerve fibre layer (pRNFL) and macular thickness between the groups.

XP patients with a known tendency for neurodegeneration were found to have a statistically significant reduction in both pRNFL (p < 0.01) and macular thickness (p < 0.001) compared with healthy controls. In contrast, there was no statistically significant difference between pRNFL and macular thickness in XP patients not expected to develop neurodegeneration compared to the same control group. When both XP groups were compared, a statistically significant reduction in total pRNFL (p = 0.02) and macular thickness (p = 0.002) was found in XP patients predisposed to neurodegeneration.

Our results suggest that pRNFL and macular thickness are reduced in XP patients with a known tendency for neurodegeneration, even before any marked neurological deficits become manifest. These findings demonstrate the potential role of retinal thickness as an anatomic biomarker and prognostic indicator for XP neurodegeneration.     

The Role of Complement Factor H in Age-related Macular Degeneration: A Review

Factor H is a 155 kDa sialic acid containing glycoprotein that plays an integral role in the regulation of the complement-mediated immune system that is involved in microbial defense, immune complex processing, and programmed cell death. These events take place primarily in fluid phase and on the cell surface and are particularly important in the context of distinguishing self from non-self. Activation of the complement system occurs within seconds and results in a proteolytic cascade eventually forming the membrane attack complex leading to cell lysis. Factor H protects host cells from injury resulting from unrestrained complement activation. Mutations and SNPs (single nucleotide polymorphisms) in Factor H have been implicated in a variety of human conditions including age-related macular degeneration (AMD), atypical hemolytic uremic syndrome, and membranoproliferative glomuleronephritis type II or dense deposit disease. It should not be surprising that these seemingly unrelated diseases involving mutations in Factor H may share common features. Because the immune process involves, in part, an inflammatory response and common or similar surface antigens, it is also not unexpected to observe features of inflammation, including deposition of bioactive complement fragments such as C3a and C5a, a cellular influx of immune related cells such as lymphocytes, and the potential for multiple organ involvement. We review recent developments in molecular genetics; SNPs, including Y402H; the three-dimensional structure; and mass spectroscopy of Factor H as it relates to the pathogenesis of eye disease. In addition, we discuss the concepts of molecular mimicry, sequestered or hidden antigens, and antigenic cross reactivity, and propose that AMD should not simply be considered to be an eye disease, but rather a systemic vascular disease where the eye has the ability to self regulate a local immune response. Identification of the initial event or inciting antigen has yet to be determined and will significantly advance the understanding of the pathogenesis of AMD.