Chloe Gottlieb

Tocilizumab for Severe Chronic Anterior Uveitis Associated with Juvenile Idiopathic Arthritis in a Pediatric Patient

Vision-threatening JIA (juvenile idiopathic arthritis)associated uveitis unresponsive to corticosteroids, immunosuppressive drugs, and anti-TNF agents is likely to require further biologic response modifier therapy to target alternate cytokines involved in the inflammatory pathways. We present one case of anterior nongranulomatous uveitis in a pediatric patient refractory to anti-TNF therapy who responded favorably to tocilizumab (TCZ), a monoclonal antibody against the interleukin-6 receptor (IL-6). A 12-year-old Caucasian boy with a history of undifferentiated JIA according to ILAR classification was referred in 2010 for progression of anterior nongranulomatous uveitis of the left eye, which was diagnosed in 2001 when he was 3 years old (3–4+ cells, no flare). The patient was both HLA-B27 and ANA positive, and had a family history that was significant for psoriasis (first-degree relative), diabetes, and Crohn disease. Manifestations of his JIA, which began in 2000, included difficulty ambulating as a result of right calf atrophy, hind foot valgus, and significant ankylosing arthritis of the right tibiotalar, subtalar, talonavicular, navicular cuneiform, and calcaneal cuboid joints assessed by MRI. He was treated with indomethacin, methotrexate (MTX), and intraarticular triamcinolone hexacetonide injections until he was treated surgically via fusion of tarsal bones in 2012. The course of uveitis prior to referral was known to be chronic (anterior chamber cells grade 1+ for greater than 3 months), and at presentation he had active anterior uveitis of the left eye (OS) per SUN criteria with 1+ cells and 1+ flare, while the anterior chamber (AC) of the right eye (OD) had no cells and no flare. Corrected visual acuity (VA) with pinhole was 20/50 OD and 20/40 OS. Complications of uveitis at the time of presentation included peripheral band keratopathy OD, epiretinal membrane (in both eyes, OU), foveal atrophy secondary to a history of macular edema OS and posterior subcapsular cataract OD with prior cataract extraction, posterior chamber intraocular lens implant (2004), and steroid-induced glaucoma OD treated with trabeculectomy (2008). Prior to referral, topical, oral, and intravitreal corticosteroids for uveitis, as well as etanercept, followed by cyclosporine A, infliximab, then adalimumab had limited effectiveness in controlling his uveitis and arthritis. With uveitis flare-ups continuing despite twice the normal dose of adalimumab (40 mg/week), the maximum dose of MTX, and inability to taper prednisone below 20 mg, the decision was made to start abatacept (April 2011; 500 mg at 0, 2, 4 weeks loading, then 500 mg/month). One month following abatacept treatment, there were 0.5+ cells AC OS and prednisone was tapered to 10 mg. Following the fourth abatacept infusion (late June 2011) the patient developed an anaphylactic reaction, which required the cessation of abatacept. Prednisone was increased to 50 mg followed by a rapid taper to 25 mg within 7 days. Following these events he developed active

Intravitreal Dexamethasone Implant for the Treatment of Macular Edema in Chronic Non-infectious Uveitis

ABSTRACT Purpose: To report observations on the single and repeat use of the dexamethasone (DEX) intravitreal implant (Ozurdex; Allergan, Inc, Irvine, CA) for the treatment of macular edema in patients with non-infectious posterior segment uveitis. Methods: A chart review of 15 consecutive patients (25 eyes) was conducted. The primary outcome measure of the first and subsequent implants was central retinal thickness (CRT) on spectral-domain optical coherence tomography (sdOCT). Secondary outcomes were best-corrected visual acuity (BCVA), time to repeat implant, and adverse events. Multilevel mixed-effects linear regression was used to determine the effect of the DEX implant compared with baseline. The Kaplan–Meier estimator was used to examine survival from relapse. Results: A total of 35 implants on 25 eyes of 15 patients were included in the analysis. Of these, 91.4% (32 of 35 eyes) had a reduction in CRT and 80% (20 of 25 eyes) had improved BCVA. After the first DEX implant, CRT decreased from 590 µm (SE: 28 µm) at baseline to 370 µm (SE: 31 µm) at 3 months (p < 0.001). The logMAR VA was 0.614 (SE: 0.089) at baseline and improved to 0.35 (SE: 0.10, p = 0.002), reaching a statistically significant difference at 3 months. A repeat implant led to VA improvement of –0.184 logMAR (SE: 0.171 logMAR) and CRT reduction of –291 µm (SE: 74 µm). There was no significant difference in effect between the first repeat implant and the initial implant. Kaplan–Meier estimates of treatment success were 72% between 3 and 6 months. Conclusions: The DEX implant is an effective adjunct treatment to systemic corticosteroid or immunomodulatory therapy. Additional research is required to determine the efficacy of DEX implant as monotherapy for controlling chronic uveitic macular edema.

Systemic Lupus Erythematosus Presenting as Acute Posterior Multifocal Placoid Pigment Epitheliopathy - Case Report and Review of the Ocular Manifestations of Systemic Lupus Erythematosus

The ocular manifestations of systemic lupus erythematosus (SLE) are numerous and may be due to complement-activating immune complex deposition causing inflammation or thrombosis, secondary effects of SLE such as hypertension, related diseases such as Sjogren’s and Antiphospholipid antibody syndrome, or a combination of these. Left untreated, these manifestations can result in serious morbidity and rarely, mortality. Here we present the first reported case of systemic lupus erythematosus masquerading as Acute Posterior Multifocal Placoid Pigment Epitheliopathy, a rare chorioretinal disorder. An overview of the numerous ocular manifestations of this disease are presented, as well as recent highlights into the pathophysiology of SLE. Case Report Acute posterior multifocal placoid pigment epitheliopathy (APMPPE) is a rare disease affecting males and females equally in their second to third decade of life. It is thought to be due to an immune driven vascular alteration of the choriocapillaris with a secondary pigment epithelial reaction. More than half are associated with a viral etiology, and up to one-third will have a viral prodrome. Systemic autoimmune diseases such as erythema nodosum, granulomatosis with polyangiitis (GPA), polyarteritis nodosa (PAN), as well as other inflammatory diseases such as cerebral vasculitis, microvascular nephropathy, scleritis and episcleritis are associated with APMPPE [1,2]. A 35 year-old Caucasian female with a known history of systemic lupus erythematosus presented with a two week history of decreased vision and headaches. She had been previously well with no symptoms of infection. Her visual acuity at presentation was 20/500 right eye and 20/30 left eye, with intraocular pressures of 12 mmHg in both eyes and no afferent pupillary defect. Diffuse anterior scleritis of the right eye was noted following instillation of phenylephrine 10%. Funduscopy revealed bilateral confluent, deep, cream-colored chorioretinal lesions of varying sizes with indistinct borders located mostly between the

Neuroretinitis with Branch Retinal Artery Occlusion in a 15-Year-Old Female

We report a case of Bartonella henselae neuroretinitis with significant disc and peripapillary edema, branch retinal artery occlusion without macula involvement and well preserved central vision. A 15-year-old female presented with loss of vision over 4 weeks in the left eye. She had a history of cat exposure, but a cat scratch, insect bite or conjunctivitis was not reported. An inferotemporal arcuate scotoma developed during the acute phase and persisted over the course of the follow-up.

Methotrexate for the treatment of noninfectious scleritis

OBJECTIVE To assess corticosteroid-sparing and inflammation control in patients with noninfectious scleritis treated with methotrexate. DESIGN Retrospective review. PARTICIPANTS Patients who received methotrexate treatment for noninfectious scleritis and who had 12 months of follow-up after treatment initiation were included in this review. METHODS The clinical records of noninfectious scleritis patients presenting at the University of Ottawa Eye Institute between September 1, 2010 and December 31, 2014 treated with methotrexate were retrospectively reviewed. Seventeen patients (21 eyes) were included in the study. Main outcome included inflammation control and corticosteroid-sparing success. Secondary outcomes were reduction of immunosuppression load and best-corrected visual acuity. RESULTS The proportion of eyes with corticosteroid-sparing success was 69.2% at 3 months and 92.3% at 12 months. The proportion of eyes that achieved inflammation control was 61.9% at 3 months and 90.5% at 12 months. The corticosteroid immunosuppression load at treatment start was 1.9 ± 2.07 and at 12 months was 0.48 ± 1.03 (p < 0.01). There was no statistically significant difference in best-corrected visual acuity. CONCLUSIONS The treatment of noninfectious scleritis with methotrexate appears to be effective at both achieving steroid-sparing success and controlling inflammation during 12 months of therapy. Immunosuppression load decreased significantly over 12 months of therapy while best corrected visual acuity was stable.

Is Acute Zonal Occult Outer Retinopathy an Autoimmune Condition? A Case Report and Literature Review

A 42-year-old woman with multiple sclerosis presented with focal decreased vision and photopsia in the left eye. Funduscopy and fluorescein angiography revealed focal chorioretinal atrophy, vascular attenuation, and bone spicules. Electroretinography revealed interocular reduction in b-wave amplitude, and Goldmann visual field perimetry studies revealed an inferior scotoma. The authors performed a literature review and conclude that the prevalence of acute zonal occult outer retinopathy in patients with autoimmune conditions may suggest that the condition is autoimmune in nature. Clinical history as well as funduscopic and retinal investigations are important in diagnosing acute zonal occult outer retinopathy.