CATASTROPHIC, BILATERAL RETINAL VASCULAR OCCLUSION AFTER INTRAVITREAL BEVACIZUMAB INJECTION.

PURPOSE: To describe two cases of catastrophic, bilateral retinal vascular occlusion after intravitreal (IVT) bevacizumab injection. METHODS: Case series. Main outcome measures included clinical and fluorescein angiography findings. RESULTS: Case 1-A 65-year-old woman with calcinosis, Raynaud phenomenon, esophageal dysfunction, sclerodactyly, and telangiectasis syndrome developed acute, severe, bilateral visual loss 2 weeks after bilateral IVT bevacizumab injection for proliferative diabetic retinopathy. Examination and fluorescein angiography revealed moderate anterior chamber inflammation, bilateral perivascular retinal hemorrhages, and near total retinal vascular occlusion. Extensive testing revealed moderately elevated anti-B2 glycoprotein (antiphospholipid) antibodies. Case 2-An 85-year-old man with polymyalgia rheumatica and left eye exudative age-related macular degeneration experienced severe, bilateral, sequential visual loss in the left eye and then right eye approximately 3 weeks after IVT bevacizumab left eye injection. Examination revealed bilateral panuveitis, diffuse perivascular exudates, and intraretinal hemorrhages. Fluorescein angiography showed diffuse venous leakage. Extensive testing revealed an elevated antinuclear antibody and mildly elevated anticardiolipin antibody. CONCLUSION: Patients with underlying retinal vascular vulnerabilities may be at increased risk of catastrophic, bilateral retinal vascular occlusion after treatment with IVT bevacizumab. The moderate-to-severe intraocular inflammation in both cases and the contralateral involvement after unilateral IVT injection in Case 2 suggest a possible delayed immune-mediated mechanism.

Bilateral acute simultaneous onset anterior uveitis presumed secondary to erlotinib: A report of two cases.

PURPOSE: To report two new cases of presumed erlotinib-associated bilateral acute simultaneous-onset anterior uveitis effectively treated with topical steroids. OBSERVATIONS: Two patients were referred to the uveitis clinic with bilateral acute simultaneous onset, anterior uveitis six weeks after starting the chemotherapeutic agent erlotinib. Frequent topical steroid were started and the inflammation responded swiftly and completely. CONCLUSIONS AND IMPORTANCE: Bilateral acute simultaneous onset anterior uveitis is a potential side effect associated with erlotinib use that has not been well described. Physicians should be aware of this potential association in patients with recent treatment with erlotinib who complain of blurred vision, photophobia, or redness of the eyes. In some cases, the inflammation responds well to topical therapy and medication can be continued.

Fundus Autofluorescence Findings in Eyes With Birdshot Chorioretinitis.

Purpose: The purpose of this study was to describe fundus autofluorescence (FAF) findings in eyes with birdshot chorioretinitis (BSCR) and to compare findings to demographic, medical, and clinical characteristics. Methods: In this multicenter, prospective, cross-sectional study, 172 eyes (86 patients) with BSCR were investigated. Participants underwent a standardized evaluation including collection of demographic data, ophthalmic and treatment history, and ophthalmologic examination. Using a standardized protocol, hypo- and hyperautofluorescence in macular and extramacular regions and specific patterns of abnormal FAF could be scored for 167 eyes. Images were scored by two independent, masked graders. Measures of visual function included best-corrected visual acuity (BCVA), contrast sensitivity (CS), color vision, and Humphrey visual field mean deviation (HVF-MD). Results: Any abnormal FAF finding was observed in 132 eyes (79.0%); macular abnormalities were observed in 84 eyes (49.1%). The most common findings were peripapillary confluent hypoautofluorescence (122 eyes [73.1%]); extramacular granular hypoautofluorescence (100 eyes [59.9%]); and macular granular hypoautofluorescence (67 eyes [40.1%]). Confluent hypoautofluorescence was related to longer median disease duration (8.7 years) than granular hypoautofluorescence (7.9 years) or hyperautofluorescence (5.6 years). Macular confluent hypoautofluorescence was associated with BCVA ≤20/25 (odds ratio [OR] = 7.83, P = 0.007), BCVA ≤20/50 (OR = 4.94, P = 0.002), and abnormal CS (OR = 4.56, P = 0.009). Presence of macular or extramacular hypoautofluorescence was related to HVF-MD ≤-3 dB (OR = 2.43, P = 0.01 and OR = 2.89, P = 0.003, respectively). Conclusions: In this large cohort, various FAF abnormalities were found, indicating that disorders of the retinal pigment epithelium are features of BSCR. Abnormal FAF is a marker of visual dysfunction in the disease.

Utility of Leflunomide in the Treatment of Drug Resistant Cytomegalovirus Retinitis.

PURPOSE: To describe leflunomide use in the treatment of drug resistant cytomegalovirus retinitis. Leflunomide has been shown to be effective in the treatment of systemic CMV viremia. METHODS: Retrospective chart review of patients with CMV retinitis treated with leflunomide. RESULTS: Two HIV-negative organ transplant recipients with UL 97 mutation resistant-genotype CMV were identified. Patient 1 developed CMV viremia post-kidney transplant and subsequently bilateral CMV retinitis. Retinitis progressed, despite intravitreal injection of ganciclovir and foscarnet, and IV foscarnet and oral valganciclovir. Retinitis control was achieved with the addition of oral leflunomide. Disease remained inactive for 22 months. Patient 2 developed CMV retinitis after lung transplant. Disease progressed despite intravitreal foscarnet injections and oral valganciclovir. Control of retinitis was achieved with addition of oral leflunomide, allowing cessation of intravitreal therapy. Disease remained inactive until his death. CONCLUSIONS: Leflunomide may be considered as a treatment option for resistant CMV retinitis.

TARANTULA HAIR ASSOCIATED PANUVEITIS TREATED WITH SUSTAINED-RELEASE INTRAVITREAL DEXAMETHASONE IMPLANT.

PURPOSE: To report a case of tarantula hair-induced panuveitis treated with sustained-release intravitreal dexamethasone implant and followed by sequential spectral domain optical coherence tomography imaging. METHODS: Findings on clinical examination, anterior segment optical coherence tomography, corneal in vivo confocal microscopy, color fundus photos, fluorescein angiography, and retinal spectral domain optical coherence tomography are presented. Sequential optical coherence tomography images demonstrated the course of the chorioretinal lesions before and after sustained-release intravitreal dexamethasone implant. RESULTS: A 19-year-old female presented with localized temporal episcleritis and scleritis that incompletely resolved despite multiple courses of topical and oral corticosteroids. She subsequently developed focal vitritis and chorioretinitis, and was found to have tarantula hair-induced panuveitis. Anterior segment optical coherence tomography and in vivo confocal microscopy confirmed the presence of tarantula hairs embedded in the anterior corneal stroma. There was only transient improvement with posterior sub-Tenon's Kenalog injection. After treatment with sustained-release intravitreal dexamethasone implant, her symptoms improved and the chorioretinal lesions became inactive. Sequential spectral domain optical coherence tomography images revealed hyperreflective inner retina lesions that progressed to involve the outer retina, and then flattened to near resolution after therapy. CONCLUSION: This is the first case showing positive clinical outcomes supported by sequential retinal spectral domain optical coherence tomography using a sustained-release intravitreal dexamethasone implant to treat ophthalmia nodosa-induced by tarantula hairs.

Choroidal Findings in Eyes With Birdshot Chorioretinitis Using Enhanced-Depth Optical Coherence Tomography.

PURPOSE: The purposes of this study were to describe choroidal findings observed using optical coherence tomography with enhanced depth imaging (EDI-OCT) in eyes with birdshot chorioretinitis (BSCR) and to test the hypothesis that these findings are related to participant demographics, clinical characteristics, and treatment. METHODS: In a multicenter, cross-sectional study, 172 eyes of 86 individuals with BSCR underwent a standardized clinical evaluation, including defined protocols for EDI-OCT imaging, with macular and peripapillary volume scans. Choroidal findings were compared to demographic information, ophthalmic examination findings, and treatment history, using logistic regression models. EDI-OCT images were evaluated by two independent, masked graders. RESULTS: Median age was 56 years old; 54 participants (62.8%) were female. One or more choroidal lesions (a predefined hyporeflective zone) were identified in 105 eyes (63.6%). Median choroidal thickness was 293 µm. Choroidal lesions were associated with longer disease durations (odds ratio [OR]: 1.08; P = 0.03), increased vitreous haze (>0.5+; OR: 4.43; P = 0.02), presence of macular edema (OR: 3.00; P = 0.02), and thick choroids (OR: 3.89; P = 0.001). Use of immunomodulatory therapy was associated with lower risk of thin choroids (lower 25th percentile, OR: 0.17; P = 0.001) or thick choroids (upper 25th percentile, OR: 0.22; P = 0.002). At least some choroidal lesions did not have corresponding, clinically apparent "birdshot lesions" on fundus examination. CONCLUSIONS: Choroidal abnormalities identified by EDI-OCT imaging are common in the macular and peripapillary regions of eyes with BSCR. Choroidal lesions were associated with clinical signs of inflammation, suggesting that they represent foci of disease activity. EDI-OCT may provide useful information about disease mechanisms and response to treatment in future, longitudinal studies of BSCR.

A new OCT finding in tuberculous serpiginous-like choroidopathy.

PURPOSE: To present a case of tubercular serpiginous-like choroiditis (SLC) with previously unreported choroidal findings on enhanced depth imaging OCT (EDI-OCT). DESIGN: Case report. METHODS: A 60-year-old female presented with decreased vision. Serpiginous choroidopathy was diagnosed. Laboratory workup revealed an infectious etiology. EDI-OCT revealed previously unreported choroidal findings. RESULTS: Laboratory workup revealed nonreactive Treponema pallidum antibodies and positive QuantiFERON Gold. CT chest showed scars of prior granulomatous disease. OCT with EDI of active lesions demonstrated infiltration of the choroid, elevation of the RPE-Bruch's membrane complex and focal increase of choroidal thickness. CONCLUSIONS: Choroidal infiltration with elevation of the RPE was demonstrated on EDI-OCT in active areas of tuberculous serpiginous-like choroiditis in this patient. This finding has not been described in imaging of patients with noninfectious serpiginous choroidopathy and may be a useful tool to differentiate serpiginous choroidopathy (SC) from serpiginous-like choroiditis (SLC). EDI-OCT may provide characterization of choroidal involvement.

Sarcoidosis: sex-dependent variations in presentation and management.

Sarcoidosis is an inflammatory disease with a wide range of clinical presentations. The manifestations and prognosis in sarcoidosis are dependent upon not only organ involvement but also age and sex. The purpose of this review is to describe the systemic and ocular manifestations of sarcoidosis with a specific focus on sex-dependent difference in presentation and management. Sarcoidosis is more common in women, particularly in patients who present after age of 50 years. Women with sarcoidosis are more likely to develop cystoid macular edema and the mortality rate is higher than that of men.

TNF inhibition for ophthalmic indications: current status and outlook.

BACKGROUND: Tumor necrosis factors (TNF) are a group of cytokines that play a role in systemic inflammation, stimulating the acute phase reaction. They are involved in systemic rheumatologic conditions such as rheumatoid arthritis and juvenile idiopathic arthritis, as well as ocular inflammatory conditions in the uveitis spectrum. Several drugs were developed to inhibit the action of TNF, thereby reducing inflammation. The three most commonly used TNF inhibitors in the US are etanercept, infliximab, and adalimumab. Newer drugs include certolizumab and golimumab. In this review, we discuss the differences in the mechanism of action, route of administration, indication, and efficacy of TNF inhibitors used in the treatment of ocular inflammation. METHODS: A review of the literature in the PubMed, MEDLINE, and Cochrane databases was conducted to identify clinical trials, comparative studies, case series, and case reports describing the use of tumor necrosis factor inhibitors in uveitis therapy. The search was limited to primary reports published in English with human subjects from 1990 to the present, yielding 5,238 manuscripts. In addition, referenced articles from the initial searches were hand searched to identify additional relevant reports. After title and abstract selection, duplicate elimination, and manual search, 69 papers were selected for analysis. Exclusion criteria included review articles and case reports on the efficacy of etanercept, infliximab, and adalimumab. Manuscripts with fewer than 20 study subjects were excluded if other larger studies existed on the use of the same drug for a particular indication. Studies with <6 months of patient follow-up were also excluded, except in the case where no other data were available. Articles meeting these criteria were then reviewed by the three authors for inclusion in this review. RESULTS: Tumor necrosis factor inhibitors have been shown to decrease inflammation associated with a number of rheumatologic conditions. Three of the five commercially available TNF inhibitors-etanercept, infliximab, and adalimumab-have been studied for their efficacy in treatment of ocular inflammation. Etanercept appears to be inadequate in controlling ocular inflammation and is not recommended for the treatment of uveitis. Infliximab and adalimumab, however, have shown encouraging results in multiple trials. Serious potential side effects such as infection, including reactivation of latent tuberculosis, malignancy, and demyelinating disease, may limit the use of TNF inhibitors in uveitis. Proper screening of patients prior to initiating these therapies may decrease these risks. DISCUSSION: Early success with infliximab and adalimumab has paved the way for new TNF inhibitors and other corticosteroid-sparing drugs to emerge in the treatment of ocular inflammation. Future studies are on the horizon to determine the long-term safety and efficacy of newer TNF inhibitors such as certolizumab and golimumab.