Jennifer E. Thorne

Multifocal choroiditis in patients with familial juvenile systemic granulomatosis

PURPOSE To document clinical features of uveitis in patients with familial juvenile systemic granulomatosis. DESIGN Retrospective chart review. METHODS Ophthalmologic examination, medical history, and clinical course in 16 patients from eight families examined at six academic medical centers. RESULTS Of the 16 patients, 15 had evidence of panuveitis with multifocal choroiditis. One patient had only an anterior uveitis. Ischemic optic neuropathy, presumably due to a small vessel vasculopathy, and retinal vasculopathy each occurred in one patient. Ocular complications were common, including cataracts in 11, glaucoma in six, band keratopathy in six, cystoid macular edema in six, and optic disk edema in six. All 16 patients had polyarthritis, and at least nine had skin rash. Often patients were misdiagnosed initially as having either juvenile rheumatoid arthritis or sarcoidosis. CONCLUSIONS Familial juvenile systemic granulomatosis is an uncommon genetic disease characterized by polyarthritis and uveitis. Panuveitis and multifocal choroiditis often may be present. Patients with a diagnosis of juvenile rheumatoid arthritis but having a family history of the disease and multifocal choroiditis should be suspected of having familial juvenile systemic granulomatosis.

Topical Nonsteroidal Anti-inflammatory Drugs and Cataract Surgery: A Report by the American Academy of Ophthalmology

OBJECTIVE To review the available evidence on the effectiveness of prophylactic topical nonsteroidal anti-inflammatory drugs (NSAIDs) in preventing vision loss resulting from cystoid macular edema (CME) after cataract surgery. METHODS Literature searches of the PubMed and the Cochrane Library databases were last conducted on January 21, 2015, with no date restrictions. The searches retrieved 149 unique citations. The first author reviewed the abstracts of these articles and selected 27 articles of possible clinical relevance for full-text review. Of these 27 articles, 12 were deemed relevant to analyze in full. Two additional articles were identified from the reference list of the selected articles, and another article was identified from a national meeting. The panel methodologist assigned ratings of level of evidence to each of the selected citations. RESULTS Nonsteroidal anti-inflammatory drug therapy was effective in reducing CME detected by angiography or optical coherence tomography (OCT) and may increase the speed of visual recovery after surgery when compared directly with placebo or topical corticosteroid formulations with limited intraocular penetration. However, the use of NSAIDs did not alter long-term (≥3 months) visual outcomes. Furthermore, there was no evidence that the benefits observed with NSAID therapy could not be obtained similarly with equivalent dosing of a corticosteroid. The reported impression that there is a pharmacologic drug synergy from the use of both an NSAID and a corticosteroid is not supported by the literature. There is no uniform method of reporting CME in the literature, which prevents accurate assessment of its incidence and response to anti-inflammatory therapies. CONCLUSIONS Cystoid macular edema after cataract surgery has a tendency to resolve spontaneously. There is a lack of level I evidence that supports the long-term benefit of NSAID therapy to prevent vision loss from CME at 3 months or more after cataract surgery. Although dosing of NSAIDs before surgery may hasten the speed of visual recovery in the first several weeks after cataract surgery, there is no evidence that this practice affects long-term visual outcomes. Standardized reporting of CME based on OCT may allow for more uniform quantitation of its incidence and more reliable assessment of treatment outcomes.

CD4 count at presentation for HIV care in the United States and Canada: Are those over 50 years more likely to have a delayed presentation?

We assessed CD4 count at initial presentation for HIV care among ≥50-year-olds from 1997-2007 in 13 US and Canadian clinical cohorts and compared to <50-year-olds. 44,491 HIV-infected individuals in the North American AIDS Cohort Collaboration on Research and Design (NA-ACCORD) were included in our study. Trends in mean CD4 count (measured as cells/mm3) and 95% confidence intervals ([,]) were determined using linear regression stratified by age category and adjusted for gender, race/ethnicity, HIV transmission risk and cohort. From 1997-2007, the proportion of individuals presenting for HIV care who were ≥50-years-old increased from 17% to 27% (p-value < 0.01). The median CD4 count among ≥50 year-olds was consistently lower than younger adults. The interaction of age group and calendar year was significant (p-value <0.01) with both age groups experiencing modest annual improvements over time (< 50-year-olds: 5[4 , 6] cells/mm3; ≥50-year-olds: 7[5 , 9] cells/mm3), after adjusting for sex, race/ethnicity, HIV transmission risk group and cohort; however, increases in the two groups were similar after 2000. A greater proportion of older individuals had an AIDS-defining diagnosis at, or within three months prior to, first presentation for HIV care compared to younger individuals (13% vs. 10%, respectively). Due to the increasing proportion, consistently lower CD4 counts, and more advanced HIV disease in adults ≥50-year-old at first presentation for HIV care, renewed HIV testing efforts are needed.

Impact of age on retention in care and viral suppression

Background:Retention in care is important for all HIV-infected persons and is strongly associated with initiation of antiretroviral therapy and viral suppression. However, it is unclear how retention in care and age interact to affect viral suppression. We evaluated whether the association between retention and viral suppression differed by age at entry into care. Methods:Cross-sectional analysis (2006–2010) involving 17,044 HIV-infected adults in 14 clinical cohorts across the United States and Canada. Patients contributed 1 year of data during their first full-calendar year of clinical observation. Poisson regression examined associations between retention measures [US National HIV/AIDS Strategy (NHAS), US Department of Health and Human Services (DHHS), 6-month gap, and 3-month visit constancy] and viral suppression (HIV RNA ⩽200 copies/mL) by age group: 18–29 years, 30–39 years, 40–49 years, 50–59 years, and 60 years or older. Results:Overall, 89% of patients were retained in care using the NHAS measure, 74% with the DHHS indicator, 85% did not have a 6-month gap, and 62% had visits in 3–4 quarters of the year; 54% achieved viral suppression. For each retention measure, the association with viral suppression was significant for only the younger age groups (18–29 and 30–39 years): 18–29 years [adjusted prevalence ratio (APR) = 1.33, 95% confidence interval (CI): 1.03 to 1.70]; 30–39 years (APR = 1.23, 95% CI: 1.01 to 1.49); 40–49 years (APR = 1.06, 95% CI: 0.90 to 1.22); 50–59 (APR = 0.92, 95% CI: 0.75 to 1.13); ≥60 years (APR = 0.99, 95% CI: 0.63 to 1.56) using the NHAS measure as a representative example. Conclusions:These results have important implications for improving viral control among younger adults, emphasizing the crucial role retention in care plays in supporting viral suppression in this population.

Therapies for macular edema associated with central retinal vein occlusion: a report by the American Academy of Ophthalmology.

PURPOSE To review the available evidence regarding the safety and efficacy of therapies for the treatment of macular edema (ME) associated with central retinal vein occlusion (CRVO). METHODS A literature search of the PubMed database was last conducted in March 2014 with no date restrictions but limited to articles published in English. A literature search of the Cochrane Library was also conducted in March 2014 with no date restrictions and without a language limitation. The combined searches yielded 108 citations, of which 20 were deemed clinically relevant for the Ophthalmic Technology Assessment Committee Retina/Vitreous panel to review in full text. Three additional studies were also identified for panel review. The level of evidence of these selected studies was reviewed by the panel methodologist. RESULTS There were 7 citations representing 4 clinical trials that provided level I evidence supporting the use of anti-vascular endothelial growth factor (VEGF) pharmacotherapies for ME associated with CRVO, including intravitreal ranibizumab (2), aflibercept (3), and bevacizumab (2). There were 3 citations representing 2 studies with level I evidence for intravitreal corticosteroid injection with dexamethasone intravitreal implant (2 citations) or triamcinolone (1 citation), although cataract and glaucoma were observed in these studies. Level I evidence is available on the limited benefit of macular grid-pattern laser photocoagulation (1 citation). Eight other citations reviewed were rated as level II, and 4 citations were rated as level III. Long-term efficacy results (≥2 years of follow-up) are limited to intravitreal ranibizumab at this time, and few studies have evaluated combination therapy with anti-VEGF and corticosteroid versus monotherapy of either class of drug. CONCLUSIONS Level I evidence indicates that intravitreal anti-VEGF pharmacotherapy is safe and effective over 2 years for ME associated with CRVO and that delay in treatment is associated with worse visual outcomes. In addition, level I evidence demonstrates short-term efficacy of intravitreal corticosteroid but also an association with a higher frequency of adverse events.

Retinopathy after long term, standard doses of hydroxychloroquine.

Editor,—While the antimalarial drug chloroquine has frequently been reported to cause retinopathy, there have been very few documented cases occurring with hydroxychloroquine (Plaquenil, Winthrop Pharmaceuticals, New York, USA).1-5Patients may tolerate large cumulative doses (up to 3923 g) of hydroxychloroquine without developing retinopathy.1 Doses of ⩽400 mg/day and ⩽6.5 mg/kg of body weight/day of hydroxychloroquine have been used safely and some authors have suggested that ophthalmic screening is not necessary for patients on these doses.3-5 We present a case of hydroxychloroquine toxicity that developed in a patient after long term use of 400 mg (6.3 mg/kg) of hydroxychloroquine daily. ### CASE REPORT A 61 year old white woman presented with a 1 year history of increased glare in both eyes without change in visual acuity. She had a history of rheumatoid arthritis for which she took hydroxychloroquine 400 …

OCULAR MANIFESTATIONS OF VASCULITIS

Systemic necrotizing vasculitis is characterized by inflammation of blood vessels and often affects the eyes. Ocular manifestations of vasculitis may involve any part of the eye or orbit. The frequency of ocular involvement generally is dependent on the size and type of blood vessels affected by the vasculitis. This article reviews the major types of ocular inflammation and then addresses the ocular manifestations of specific systemic vasculitides.

Choroidal white lesions as an early manifestation of sarcoidosis.

Purpose: To describe the natural history of a series of patients with fine white choroidal lesions and uveitis of previously unknown cause. Methods: A retrospective chart review of 11 patients with chronic uveitis and multiple, small (50‐100μm), peripheral while lesions of the choroid was performed using a stan‐ dardized questionnaire form. Results: Ten of 11 patients were white women with an average age of 62 years. Seven of 11 patients had panuveitis; 4 of 11 patients had vitritis; and 6 of 11 patients had cystoid macular edema. Choroidal white lesions were bilateral in all but one patient. Seven of 11 patients were followed up for more than 1 year. During a 12‐ to 173‐month follow‐up (mean, 94 months), these patients showed coalescence and atrophy of the white lesions. Initial systemic examination for the cause of the uveitis and white choroidal lesions was negative in all seven patients. With long‐term follow‐up, sarcoidosis was diagnosed in five of the seven patients followed for more than 1 year. Conclusions: The pattern of inflammatory white choroidal lesions distributed in the peripheral retina that atrophy and coalesce with time and that are associated with uveitis in middle‐aged white women may represent an early form of sarcoidosis.

Caught by a masquerade: Sclerosing orbital inflammation

Idiopathic sclerosing inflammation of the orbit is a distinct form of orbital inflammatory disease characterized by slow and relentless involvement of orbital structures. It is this insidious and relentless course that makes distinction from neoplastic lesions clinically difficult. We report the case of a patient with a several-week history of headache and decreased vision that was originally thought to represent an optic nerve sheath meningioma, based on clinical and radiographic evaluation. Subsequent histopathology from an optic nerve biopsy, however, was more consistent with optic nerve glioma. Eventually, pathologic examination of whole sections through the optic nerve was required to establish and confirm the actual diagnosis of sclerosing orbital inflammation.

Magnetic resonance imaging of acquired brown syndrome in a patient with psoriasis

PURPOSE To report the occurrence of acquired Brown syndrome and associated magnetic resonance imaging findings in a patient with psoriasis. METHODS A 42-year-old woman with a history of psoriasis developed pain, double vision, and limited elevation of her left eye in adduction. An orbital magnetic resonance image with gadolinium enhancement was obtained. RESULTS Orbital magnetic resonance image disclosed abnormal enhancement of the left trochlea/tendon complex. The patients symptoms resolved with corticosteroid therapy. CONCLUSIONS Acquired Brown syndrome may be associated with psoriasis. The inflammation of the trochlea/tendon complex that can cause acquired Brown syndrome can be demonstrated on magnetic resonance image.