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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.

To compare the frequencies of attacks before and after immunomodulatory therapy (IMT) with mycophenolate mofetil (MMF) in recurrent punctate inner choroidopathy (PIC) and to report fundus autofluorescence (FAF) findings.Eight patients who had at least two recurrent episodes of increased activity before MMF and 12 months of documented clinical course before and after MMF were included. The frequencies of attacks before and after MMF were compared. FAF images evaluated.Before MMF, the 8 patients experienced 19 attacks of recurrent disease activity; during MMF therapy, 3 (38%) patients experienced 6 recurrent episodes. The attack frequencies were 1.09 ± 0.75 before and 0.23 ± 0.32 during treatment (p = .036). Among the 6 patients who had FAF, surrounding hyperautofluorescent halo was detected in 4 and recurrence occurred in 2 who did not show any changes on the intensity during treatment.In the index study, the employment of MMF has decreased frequency of attacks in recurrent PIC. FAF may be employed to monitor and predict the response to treatment.

Diabetes mellitus is a growing global epidemic. Patients with this disease present with a variety of health conditions, including a number of ocular complications that threaten vision, such as proliferative diabetic retinopathy and macular edema. Diabetic papillopathy, another potential ocular complication from diabetes, is a self-limiting, sometimes bilateral disease that may affect both type 1 and type 2 diabetics. It is characterized by optic disc swelling caused by vascular leakage and axonal edema in and around the optic nerve head. Occasionally, it may be accompanied by intraretinal hemorrhages and hard exudates. Diabetic papillopathy tends to be mild and is usually associated with good visual prognosis; however, there are some cases in which permanent visual impairment can develop. The pathogenesis remains largely unknown, but there has been evidence suggestive of its associations with a small cup/disc ratio and rapid reduction in glycemia. There is no validated therapy for diabetic papillopathy; however, current case reports have shown promising results after local injections of corticosteroids as well as bevacizumab (Avastin), a potent monoclonal antibody that has been employed for the treatment of ocular vaso-proliferative diseases such as choroidal neovascular membranes associated with age-related macular degeneration and proliferative diabetic retinopathy.

Purpose To compare the effectiveness and safety of the combination therapy of rituximab (RTX) and intravenous immunoglobulin (IVIg) to other immunosuppressive regimens in the treatment of ocular cicatricial pemphigoid (OCP). Design Retrospective, comparative, interventional case series. Participants Twelve patients with OCP. Methods We reviewed medical records of 12 patients with OCP. Ten of the 12 patients were blind in 1 eye after initial systemic immunosuppressive therapies (phase 1 treatment). The patients were then divided into 2 groups based on treatments received during phase 2. The study group consisted of 6 patients who received the combination of RTX and IVIg during phase 2 of their treatment. For comparison purposes, the control group consisted of 6 patients who during phase 2 of their treatment received more aggressive immunosuppressive therapies, but not RTX and IVIg, because the insurance carriers refused to pay for the combination therapy. Main Outcome Measures Blindness (best-corrected visual acuity [BCVA] ≤20/200) and OCP staging (Foster). Results The median total follow-up periods were 57.5 and 55.5 months in the control group and the study group, respectively. After phase 1 treatment, all 6 patients in the control group were blind in 1 eye. Similarly, 4 of the patients in the study group were blind in 1 eye, whereas 2 had good BCVA bilaterally but experienced persistent conjunctival inflammation despite phase 1 treatment. After phase 2 treatment, all 6 patients in the control group had OCP progression and became blind in both eyes. In contrast, BCVA was stable and no further progression of OCP staging was observed in all 6 patients in the study group. In the study group, the median follow-up from completion of the RTX and IVIg treatment protocol was 11 months. No adverse events, immediate or delayed, were reported in any of the patients who received the combination therapy of RTX and IVIg. Conclusions In this preliminary study, the combination therapy of RTX and IVIg arrested disease progression and prevented total blindness in patients with recalcitrant OCP. Financial Disclosure(s) The authors have no proprietary or commercial interest in any of the materials discussed in this article.

PURPOSE To describe a patient with sudden unilateral loss of vision associated with white inner retinal lesions and a history of a nonspecific viral infection. METHODS Case report. We reviewed the medical record and retinal angiogram of one patient. RESULTS The patient presented with a sudden loss of visual acuity in the left eye. Fundus examination revealed multiple white inner retinal lesions and hyperemic optic disks in both eyes. Spontaneous improvement of visual acuity and resolution of the retinal lesions were noted over the ensuing weeks. CONCLUSION Retinitis can present as a nonspecific response to various infectious agents. We herein report a case of acute multifocal inner retinitis associated with a nonspecific viral illness.

To report two cases of spontaneous Retisert implant dissociation with dislocation of the medication reservoir into the anterior chamber.Case reports.Two patients with chronic, noninfectious uveitis following Retisert implantation between 6 and 7 years prior presented with a complaint of a "white spot" in their right eyes. Both patients had previous pars plana vitrectomies. Anterior segment examination revealed a dislocated medication reservoir of the Retisert implant in the inferior portion of anterior chamber with associated reservoir-corneal endothelial touch and stromal edema. The reservoirs were subsequently retrieved via a pars plana approach and removed from the anterior chamber through a corneal incision.Spontaneous dissociation of the Retisert implant with dislocation of the medication reservoir into the anterior chamber can be a late complication of Retisert implantation. Eye care professionals and patients should be aware of this complication, particularly with long-term intraocular retention of this device.

Background Dermatomyositis is rare during pregnancy and, if untreated, is associated with poor fetal outcome. Corticosteroids are a standard treatment for dermatomyositis in pregnancy, but they have adverse effects. Intravenous immune globulin is an effective therapy for this condition and may have few adverse effects. Case A young, white primigravida presented with dermatomyositis at 4 5/7 weeks of gestation (creatine kinase 2,762 units/L). Intravenous immune globulin was administered monthly at a dose of 1 g/(kg.d) for 2 consecutive days. The patient's symptoms resolved and no complications were experienced during therapy. At term, creatine kinase was 29 units/L and a healthy 3,657.5-g (8-lb, 1-oz) neonate was born. Conclusion Pregnant patients with dermatomyositis can be treated with intravenous immune globulin, resulting in good fetal outcome, thus avoiding the deleterious effects of corticosteroid therapy on pregnancy.

Purpose To report on a patient with congenital erythropoietic porphyria who presented with scleral necrosis. Method Case report. Results A 34-year-old man with a long history of congenital erythropoietic porphyria was referred to us for evaluation of necrotizing scleritis of the right eye. The patient presented with a 3-month duration of eye pain and redness, which initially responded to oral and topical corticosteroids. However, upon corticosteroid taper, the symptoms quickly recurred. The patient was initially tried on oral azathioprine therapy, which failed to induce resolution of the symptoms. Full serological investigation did not suggest that the necrotic process was secondary to an immunologically driven process, and we proceeded with scleral biopsy and patch graft. The biopsy disclosed atrophic changes of the sclera with calcium-like plaques, without evidence of inflammation. Aggressive strategy of sun avoidance was implemented and immunosuppressive therapy discontinued. One year after the surgery, the patient remains asymptomatic without recurrence of ocular disease. Conclusions This case highlights the usefulness of tissue biopsy in porphyria when one is uncertain as to whether a necrotizing process is driven by inflammation or phototoxic damage. When the underlying mechanism is clearly identified, the appropriate therapy can then be instituted to prevent further damage.

Adamantiades-Behçet’s disease (ABD) is a multisystemic vasculitic disease. It is most prevalent in the Eastern Mediterranean countries and the Eastern region of Asia. Its effect on the eye can range from mild to debilitating, resulting in total blindness. A necrotizing and obliterative vasculitis affects both arteries and veins of organs. Recurrent attacks of uveitis, oral aphthous ulcers, skin lesions, and genital ulcers are common. Topical and systemic corticosteroids have been the mainstay in the treatment of ocular inflammation for many years; however, due to the several known side effects of corticosteroids and thanks to scientific advances, more novel approaches to ABD treatment have been emerging. Antimetabolites such as methotrexate and azathioprine have been utilized with the latter showing positive results. Chlorambucil has been utilized effectively for ocular manifestations of ABD. Interferon alpha has shown encouraging results in the management of refractory ocular inflammation associated with ABD, either alone or in combination with other immunosuppressive agents. Surgical interventions to deal with complications from ABD can be safely done if adequate control of inflammation is achieved peri-operatively. Early detection and aggressive treatment, when needed, have proven to be essential in the management of this relentlessly explosive disease.

To compare the effectiveness and safety of the combination therapy of rituximab (RTX) and intravenous immunoglobulin (IVIg) to other immunosuppressive regimens in the treatment of ocular cicatricial pemphigoid (OCP).Retrospective, comparative, interventional case series.Twelve patients with OCP.We reviewed medical records of 12 patients with OCP. Ten of the 12 patients were blind in 1 eye after initial systemic immunosuppressive therapies (phase 1 treatment). The patients were then divided into 2 groups based on treatments received during phase 2. The study group consisted of 6 patients who received the combination of RTX and IVIg during phase 2 of their treatment. For comparison purposes, the control group consisted of 6 patients who during phase 2 of their treatment received more aggressive immunosuppressive therapies, but not RTX and IVIg, because the insurance carriers refused to pay for the combination therapy.Blindness (best-corrected visual acuity [BCVA]or =20/200) and OCP staging (Foster).The median total follow-up periods were 57.5 and 55.5 months in the control group and the study group, respectively. After phase 1 treatment, all 6 patients in the control group were blind in 1 eye. Similarly, 4 of the patients in the study group were blind in 1 eye, whereas 2 had good BCVA bilaterally but experienced persistent conjunctival inflammation despite phase 1 treatment. After phase 2 treatment, all 6 patients in the control group had OCP progression and became blind in both eyes. In contrast, BCVA was stable and no further progression of OCP staging was observed in all 6 patients in the study group. In the study group, the median follow-up from completion of the RTX and IVIg treatment protocol was 11 months. No adverse events, immediate or delayed, were reported in any of the patients who received the combination therapy of RTX and IVIg.In this preliminary study, the combination therapy of RTX and IVIg arrested disease progression and prevented total blindness in patients with recalcitrant OCP.

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