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Objective To compare the demographic, clinical, ancillary testing, and multimodal imaging characteristics of birdshot chorioretinopathy (BSCR) patients with late recurrence and birdshot patients with durable remission. Patients and Methods This was a retrospective observational case series. The above-mentioned parameters were studied in BSCR patients with late recurrence (group 1) and BSCR patients with durable remission (group 2). Results Fifty-five patients were included in this study. The average age of patients was 62.1 ± 11.1 years (range, 35–88 years). Groups 1 and 2 included 20 (36.4%) and 35 (63.6%) patients, respectively. In group 1, the average age of patients was 60.5 ± 10.39 years (range, 35–79 years). The female-to-male ratio was 16:4. In group 2, the average age of patients was 63.1 ± 11.6 years (range, 37–88 years). The female-to-male ratio was 22:13. None of the demographic, clinical, ancillary testing, and multimodal imaging parameters were statistically significantly different between the two groups. Using a receiver operating characteristics (ROC) curve, we found that the ideal duration of successful therapy to induce durable remission was 30 months with 70% sensitivity and 40% specificity (ideal point on the curve). A Kaplan–Meier survival curve demonstrated that late recurrence was seen within 30 months after stopping successful treatment of patients with BSCR. Conclusion There are no demographic, clinical, ancillary testing, or multimodal imaging characteristics that can predict late recurrence in BSCR patients. However, we found that 30 months of successful treatment may be ideal and recommended.

The purpose of this review was to investigate the idea that inflammatory events of the conjunctiva and ocular surface may act as triggering events for the onset of ocular mucus membrane pemphigoid (oMMP).A retrospective chart review of patients with biopsy-proven oMMP and no systemic pemphigoid disease. The presence, or absence, of the following inflammatory conditions at the time of OMMP diagnosis was noted: significant eyelid disease, significant atopic eye disease, Stevens-Johnson syndrome, graft-versus-host disease, viral keratitis, sarcoidosis with ocular involvement, chemical burns, medicamentosa, Sjogren syndrome, systemic lupus erythematosus with ocular involvement, and epidemic keratoconjunctivitis. Response to immunomodulatory therapy (IMT) was also recorded.A total of 779 patient records were identified. Conjunctival biopsy was present in 724 patients, with 646 (89.2%) being positive. One hundred thirty-nine patients (21.5%) with positive biopsies had extraocular pemphigoid disease and were excluded from further analysis. Of the 507 included patients, 154 (30.4%) had at least one of the specified inflammatory conditions present at the time of OMMP diagnosis. One hundred eighteen patients (23.3%) had only 1 such condition, 35 (6.9%) had 2, and 1 patient had 3. In patients with at least one of these conditions present, response to IMT was seen in 84.9% of patients with sufficient follow-up.Our study suggests that oMMP may arise as a secondary pathology to acute inflammatory events or chronic inflammatory states of the conjunctiva and ocular surface.

Purpose To report a case of systemic and ocular toxoplasmosis in an immunocompetent patient, who developed myocarditis with resulting cardiogenic shock and multiple organ failure, followed by bilateral panuveitis masquerading as endogenous endophthalmitis. Methods Single case report with images. Results A 59-year-old man with a history of monoclonal gammopathy of undetermined significance and associated scleromyxedema but otherwise immunocompetent was admitted to the intensive care unit for cardiogenic shock and multiple organ failure due to presumed viral myocarditis. After hospital discharge, ophthalmic examination revealed what seemed to be endogenous fungal endophthalmitis in both eyes. The ocular inflammation failed to improve on local and systemic antifungal therapies. After repeated testing and vitrectomy, the causative organism responsible for his intraocular inflammation remained elusive. The patient was then found to have significantly elevated serum titers of anti-Toxoplasma gondii IgG and IgM, followed by an appearance of a focal retinochoroidal lesion more typical of ocular toxoplasmosis. Systemic anti-Toxoplasma therapy led to resolution of intraocular inflammation, and the patient had since fully recovered from the myocarditis and its multiple comorbidities. Of note, myocardial biopsy and polymerase chain reaction testing of aqueous and vitreous fluids were all negative for Toxoplasma. Conclusion Even in an immunocompetent patient, Toxoplasma can result in myocarditis with significant morbidities and even death, and its ocular manifestation may be quite different from the classic focal retinochoroiditis. In addition, polymerase chain reaction analysis of ocular fluids can be unreliable in an immunocompetent host, and thus treatment decision should be guided by clinical history and examination findings.

Background/purpose To describe the use of topical mitomycin-C in sclerostomy revision for recalcitrant idiopathic uveal effusion syndrome. Methods A 50-year-old healthy man presented with painless, gradual vision loss in the right eye. He underwent multimodal retinal imaging with wide-field fundus photography, spectral domain optical coherence tomography, and B-scan and A-scan ultrasonography. He was found to have idiopathic (non-nanophthalmic) uveal effusion syndrome with choroidal and serous retinal detachments in the right eye and a peripheral choroidal detachment in the left eye. Central vision became threatened in the right eye. Medical treatment with oral corticosteroids and surgical treatment with choroidal drainage through sclerostomies and sclerostomy revision were administered. Results Initial treatment with systemic corticosteroids was ineffective. Subsequent choroidal drainage through sclerostomies only partially resolved the effusion. Later sclerostomy revision with application of topical mitomycin-C led to complete resolution with anatomical stability maintained after at least 42 months of follow-up. Conclusion Successful use of topical mitomycin-C in sclerostomy revision has not previously been reported in idiopathic (non-nanophthalmic) uveal effusion syndrome. We propose that topical mitomycin-C may be considered as a potential therapeutic adjunct in the treatment of refractory idiopathic uveal effusion syndrome before further sclerostomy procedures are attempted in additional quadrants of the eye.

To search findings that can explain the heterogeneity between Resistant and Responsive patients with birdshot chorioretinopathy.This was a retrospective observational case series on "Responsive" versus "Resistant" birdshot chorioretinopathy.One-hundred-eighty and Ninety-nine patients were included in the Responsive and Resistant groups respectively. Multivariate analysis of paraclinical variables at the first visit demonstrated that mean deviation (p = .04), pattern standard deviation (p.001), optic nerve head leakage (p = .012), large vessel leakage and staining (p = .01), and macular small vessel leakage (p = .03) were statistically significantly different between the two groups; however, at the visit preceding successful therapy, only macular small vessel leakage (p = .01) was statistically significantly different between the two groups..Small vessel leakage in the macular area and/or optic nerve head leakage at the earliest visit might be risk factors for resistant birdshot chorioretinopathy.

To evaluate the effect of repository corticotropin injection (RCI) on regulatory T cell population in patients with noninfectious retinal vasculitis.Patients with active noninfectious retinal vasculitis were included in a prospective nonrandomized open-label study.Eighteen patients (33 eyes) were included in the study. Eleven (61.1%) patients [20 (60.6%) eyes] and 7 (38.9%) patients [13 (33.3%) eyes] were in the responsive and non-responsive groups, respectively. We did not find any statistically significant difference within the PPP-R group, within the PPP-NR group, or between these two groups in regard to regulatory T cell population. No significant systemic or ocular complications were found.RCI may be a complementary treatment in patients with non-infectious retinal vasculitis with or without uveitis. This study did not demonstrate an increase in regulatory T cell population in patients with noninfectious retinal vasculitis.

To determine the efficacy and safety of intravenous tocilizumab in refractory juvenile idiopathic arthritis associated uveitis.This was a retrospective observational case series. Patients with refr...

Purpose To report a case of acute macular neuroretinopathy (AMN) after intravitreal triamcinolone acetonide (TRIESENCE®) injection for cystoid macular edema secondary to birdshot chorioretinopathy. Method A case report. Patient A 62-year-old female. Results The patient presented with acutely decreased vision and a ring scotoma around her central vision three days after intravitreal triamcinolone acetonide (TRIESENCE®) injection for cystoid macular edema in her right eye (OD) secondary to birdshot chorioretinopathy. She had undergone pars plana vitrectomy, cataract extraction, and secondary intraocular lens implantation OD three months prior to the recent injection. Best-corrected visual acuity (BCVA) was 20/1000 OD and 20/50 OS. Intraocular pressure was 21 mmHg OD and 12 mmHg OS. Fluorescein angiography demonstrated a hypofluorescent area in the perifoveal zone OD. Optical coherence tomography OD depicted hyperreflective areas in the outer nuclear layer, outer plexiform layer, and retinal pigment epithelium. We diagnosed her with AMN OD and started her on brimonidine three times a day OD. She came back a week later with resolved scotoma and her vision improved to 20/60 OD. Five weeks later, BCVA was 20/40 and Intraocular pressures (IOP) was 12 mmHg OD. Conclusions and importance Intravitreal triamcinolone injection may be a cause of AMN with cystoid macular edema (CME) and borderline-high intraocular pressure. Brimonidine may be an effective treatment for these patients in the early course of the disease.

Purpose: To show whether subcutaneous repository corticotropin injection (RCI, Acthar® Gel, a repository corticotropin injection, can be an effective potential therapeutic agent for noninfectious retinal vasculitis. Methods: Patients with active retinal vasculitis were followed with serial ultra-wide-field fluorescein angiograms and treated with 80 units of subcutaneous repository corticotropin injection twice weekly. Results: Primary outcome of ≥ 50% improvement in response level (RL) for retinal vasculitis and percent improvement in retinal vasculitis severity scoring (RVSS) by more than one quartile ( ≥ 25%) at week 12 was met in 15 and 16 of the 30 total eyes, respectively, including 1 eye with severe retinal vasculitis in each group. Complete resolution of retinal vasculitis was seen in seven eyes with a mean time of 17.1 weeks. Intraocular pressure elevation requiring therapy and cataract progression were noted in two and three eyes, respectively. One patient stopped medication due to side effects (injection site reaction). Conclusion: Repository corticotropin injection was well-tolerated overall. Repository corticotropin injection may be an effective therapeutic agent in the treatment of noninfectious retinal vasculitis.

Purpose: To evaluate the efficacy of intravenous methotrexate and methylprednisolone in severe, sight-threatening ocular inflammatory conditions.Methods: This was a retrospective observational case...

To evaluate the wide-field fundus fluorescein angiography (WFA) characteristics of uveitis associated with juvenile idiopathic arthritis (JIA-uveitis). Retrospective review of records. WFA with Spectralis (Heidelberg) of JIA-uveitis patients were analyzed using the scoring system by Angiography Scoring for Uveitis Nomenclature. Thirty-seven eyes of 20 patients were studied. A total score of at least 1 was noted in 27 eyes (72.97%). WFA features included optic disc hyperfluorescence (51.35%), macular leakage (27.03%), retinal vascular staining/leakage at posterior pole (27.03%) and peripheral retina (64.86%), capillary leakage at the posterior pole (37.84%), and peripheral retina (59.46%). A decision to change the management plan was made in 8 of 9 patients with bilateral quiet anterior chambers after WFA results. More than 70% of JIA-uveitis eyes showed some WFA-evidence of posterior segment inflammation, which changed the course of therapy for a major proportion of patients with no clinically active anterior chamber inflammation.

Ocular surface inflammation severe enough to cause cicatrizing changes in conjunctiva may ensue from a wide variety of pathologies, including noninfectious, infectious, and masquerade-type processes. Eventually uncontrolled disease leads to blindness via secondary corneal ulceration, scarring, and neovascularization. These entities often appear similar in their progression or may even co-exist, making management difficult. Conjunctival biopsy is an important tool in helping the physician to better distinguish between different pathologies. Proper technique and handling are critical to maximizing sensitivity as well as implementation of various means of immunoreactant staining when necessary, i.e., direct immunofluorescence and immunoperoxidase technique. Medical therapy may then be targeted to the specific disease process. Surgical management is reserved for vision rehabilitation and ideally performed when inflammation is in remission.