Long-term, drug-free remission of sympathetic ophthalmia with high-dose, short-term chlorambucil therapy.

OBJECTIVE: To evaluate the safety and effectiveness of short-term, high-dose chlorambucil therapy in achieving long-term, drug-free remission in the treatment of sympathetic ophthalmia (SO). DESIGN: Retrospective case series. PARTICIPANTS: Sixteen patients with SO treated with high-dose, short-term chlorambucil therapy between 1970 and 2010. METHODS: Descriptive and bivariate analyses were used to characterize disease and outcomes. MAIN OUTCOME MEASURES: Months of disease-free remission, prevalence rate of relapse, and prevalence of serious treatment-related adverse events. RESULTS: Sixteen patients with SO treated with short-term, high-dose chlorambucil were identified. Patients were treated with chlorambucil for a median of 14.0 weeks (mean, 14.5 weeks; range, 12.0-19.0 weeks). Median follow-up was 98.5 months (mean, 139.1 months; range, 48-441 months) from initiation of chlorambucil therapy. Control of inflammation was achieved in 100% of patients. Thirteen patients (81.3%) maintained vision of 20/40 or better in the sympathizing eye. Four patients (25%) relapsed after a median of 83 months (mean, 131 months) after cessation of systemic therapy. Seventy-five percent of relapses were controlled with topical therapy only. Conjunctival Kaposi's sarcoma developed in 1 patient. No patient demonstrated systemic malignancy. CONCLUSIONS: Short-term, high-dose chlorambucil therapy provides sustained periods of drug-free remission. With median follow-up of more than 8 years (mean, 11.6 years; range, 4-37 years), there was a low rate of recurrence and minimal long-term serious health consequences or adverse events. Because SO may be a lifelong condition and because chlorambucil therapy may offer long-term, drug-free remission, this treatment may be worth considering early in the decision-making process for severe sight-threatening disease.

The Humira in Ocular Inflammations Taper (HOT) Study.

PURPOSE: To assess factors that impact the risk of relapse in patients with noninfectious uveitis (NIU) who undergo adalimumab tapering after achieving remission. DESIGN: Retrospective study. METHODS: In this multicenter study, patients with NIU were treated with adalimumab and subsequently tapered. Patient demographics, type of NIU, onset and duration of disease, the period of inactivity before tapering adalimumab, and the tapering schedule were collected. The primary outcome measures were independent predictors of the rate of uveitis recurrence after adalimumab tapering. RESULTS: Three hundred twenty-eight patients were included (54.6% female) with a mean age of 34.3 years. The mean time between disease onset and initiation of adalimumab therapy was 35.2 ± 70.1 weeks. Adalimumab tapering was commenced after a mean of 100.8 ± 69.7 weeks of inactivity. Recurrence was observed in 39.6% of patients at a mean of 44.7 ± 61.7 weeks. Patients who experienced recurrence were significantly younger than those without recurrence (mean 29.4 years vs 37.5 years, P = .0005), and the rate of recurrence was significantly higher in younger subjects (hazard ratio [HR] = 0.88 per decade of increasing age, P = .01). The lowest rate of recurrence was among Asian subjects. A faster adalimumab taper was associated with an increased recurrence rate (HR = 1.23 per unit increase in speed, P < .0005). Conversely, a more extended period of remission before tapering was associated with a lower rate of recurrence (HR = 0.97 per 10-weeks longer period of inactivity, P = .04). CONCLUSIONS: When tapering adalimumab, factors that should be considered include patient age, race, and duration of disease remission on adalimumab. A slow tapering schedule is advisable.

Association Between Ocular Trauma and Activation of Ocular Toxoplasmosis.

PURPOSE: To evaluate the association between ocular trauma and activation of ocular toxoplasmosis. METHODS: Retrospective review of 686 patients with ocular toxoplasmosis and its association with trauma to the eye or the head within 1 week of activation. RESULTS: Ten patients with a history of trauma and activation of ocular toxoplasmosis were detected (10/686; 1.45%). Nine patients showed a primary focus of retinitis without a previous scar and one patient had a recurrent form of ocular toxoplasmosis. From these 10 patients, Toxoplasma IgG was positive in eight of them. The median age of the patients was 35.8 years-old (range 17 to 65). CONCLUSIONS: These cases suggest that trauma can be associated with activation of retinal bradyzoite cysts in ocular toxoplasmosis.

Superficial retinal precipitates in patients with syphilitic retinitis.

PURPOSE: The purpose of this study was to describe the occurrence of superficial retinal precipitates in patients with syphilitic retinitis. METHODS: This was a retrospective, observational case series of nine eyes of eight patients with syphilitic retinitis associated with superficial retinal precipitates. The clinical, photographic, angiographic, and laboratory records were reviewed. Characteristics and treatment response of these superficial retinal precipitates were observed. RESULTS: All patients were Caucasian men, including 5 men who have sex with men (62.5%) and 6 (75.0%) who were positive for human immunodeficiency virus. None of the patients were previously diagnosed with syphilis. All patients developed panuveitis and a distinctly diaphanous or ground-glass retinitis associated with creamy yellow superficial retinal precipitates. In 3 patients (37.5%), the retinitis had a distinctive wedge-shaped appearance. Five patients (62.5%) had associated retinal vasculitis, 3 (37.5%) had serous retinal detachment, 2 (22.2%) had intraretinal hemorrhage, and 2 (22.2%) had papillitis. Within 2 weeks of initiating intravenous penicillin treatment, 7 patients (87.5%) experienced visual recovery to >or= 20/40. All affected eyes showed rapid resolution of clinical signs with minimal alternations of the retinal pigment epithelium in areas of prior retinitis after completion of antibiotic therapy. CONCLUSION: Characteristic superficial retinal precipitates may occur over areas of syphilitic retinitis. Improved recognition of this highly suggestive clinical sign may aid in early diagnosis and treatment.

Clinical Spectrum of HLA-B27-associated Ocular Inflammation.

Human leukocyte antigen (HLA)-B27-associated anterior uveitis (AU) is the most commonly diagnosed form of AU and represents the largest entity of non-infectious uveitis around the world. The most typical ocular manifestation associated with HLA-B27 consists of unilateral AU of acute onset. The HLA-B27-associated acute AU represents a distinct clinical entity occurring typically in young adults between the ages of 20 and 40 years. HLA-B27-associated acute AU is typically unilateral and lasts usually several weeks and diminishes within 3 months in the majority of patients. The anterior chamber shows typically severe cellular reaction and flare, as well as a fibrinous exudate. Frequently, posterior synechiae are formed and occasionally hypopyon is present. The pattern of the disease is recurrent with a full remission between the attacks. Intraocular pressure during active periods is typically low due to inflammation of ciliary body and decreased aqueous production. Less typical presentations are also recognized and include the development of chronic inflammation, posterior segment involvement, episcleritis, and scleritis. An isolated retinal vasculitis in HLA-B27-positive patients may develop, mostly in those with inflammatory bowel disease. Chronic AU, which may be either unilateral or bilateral affects up to 20% of patients. Ocular complications of HLA-B27-associated AU are diverse and include commonly posterior synechiae, cataract, glaucoma and/or hypotony. The visual outcome and complications of HLA-B27-associated AAU are frequently being compared with HLA B27-negative patients with AU and show that the prognosis of HLA-B27-associated uveitis is rather favorable, as <2% developed legal blindness and <5% visual impairment. A novel algorithm called the "Dublin Uveitis Evaluation Tool (DUET)" has been proposed to guide ophthalmologists to refer appropriate HLA-B27-positive patients with uveitis to rheumatologists.

Subretinal abscess due to Nocardia farcinica resistant to trimethoprim- sulfamethoxazole in a patient with systemic lupus erythematosus.

PURPOSE: To report a case of subretinal abscess due to Nocardia farcinica resistant to trimethoprim-sulfamethoxazole in a patient with systemic lupus erythematosus on immunosuppressive therapy. DESIGN: Observational case report. METHODS: We retrospectively studied the medical record of a patient with nocardiosis. RESULTS: The microorganism disseminated from the lungs (pneumonia) to the eye and brain. The ocular lesion appeared to be a yellowish, lobulated subretinal abscess with irregular surface and superficial retinal hemorrhages. As it was not responding to empiric therapy for nocardia, pars plana vitrectomy and aspiration of the subretinal material was performed to confirm the etiology. CONCLUSION: In an immunocompromised patient with pulmonary involvement and a subretinal abscess with a characteristic aspect, one should consider nocardia as a possible etiology taking into account its possible antibiotic resistances.

Propionibacterium acnes endophthalmitis with bacterial sequestration in a Molteno's implant after cataract extraction.

PURPOSE: To report a case of Propionibacterium acnes endophthalmitis following uncomplicated cataract extraction with bacterial sequestration in a preexisting Molteno's drainage implant. DESIGN: Interventional case report. METHODS: A 7-year-old girl with congenital glaucoma and a preexisting Molteno's drainage implant developed anterior nongranulomatous uveitis 4 months following cataract surgery. P. acnes endophthalmitis was diagnosed by polymerase chain reaction, Southern blot, and electron microscopy. RESULTS: Extraction of the Molteno's implant was required to control the persistent intraocular inflammation and to convert the results of polymerase chain reaction and Southern blot testing of aqueous sample for P. acnes from positive to negative. CONCLUSION: P. acnes may be sequestered in glaucoma implants, potentially requiring implant removal to treat cases of P. acnes endophthalmitis.

Review for disease of the year: differential diagnosis of ocular toxoplasmosis.

The diagnosis of ocular toxoplasmosis is mainly clinical, based in the presence of focal necrotizing retinochoroiditis often associated with a preexistent chorioretinal scar, and variable involvement of the vitreous, retinal blood vessels, optic nerve, and anterior segment of the eye. Recognition of this clinical spectrum of toxoplasmic retinochoroiditis is crucial, but other infectious, noninfectious, and neoplastic entities should also be considered in the differential diagnosis. Investigations such as serological tests, polymerase chain reaction of ocular fluids, and assessment of intraocular antibody synthesis are helpful in uncertain cases. This article provides an overview of the differential diagnosis of ocular toxoplasmosis, focusing on the most important entities to be considered and emphasizing distinctive features of each one of them in the clinical setting. Ocular toxoplasmosis has multiple clinical manifestations, which partially overlap with those of other entities and these should be carefully considered when making the differential diagnosis, particularly in less typical cases.

Intraocular inflammation associated with ocular toxoplasmosis: relationships at initial examination.

PURPOSE: To describe characteristics of intraocular inflammation in eyes with active ocular toxoplasmosis and to identify relationships between signs of inflammation, complications (including elevated intraocular pressure [IOP]), other disease features, and host characteristics. DESIGN: Multicenter, retrospective, cross-sectional study. METHODS: We reviewed the medical records of 210 patients with toxoplasmic retinochoroiditis at seven international sites (North America, South America, and Europe) for information from the first examination at each site during which patients had active retinal lesions. Signs of inflammation included anterior chamber (AC) cells and flare and vitreous humor cells and haze. Retinal lesion characteristics included size (< or =1 disc area [DA] or >1 DA) and presence or absence of macular involvement. RESULTS: AC cells and flare were related to vitreous inflammatory reactions (P < or = .041). One or more signs of increased inflammation were related to the following factors: older patient age, larger retinal lesions, and extramacular location. In 30% of involved eyes, there was evidence of elevated IOP (despite use of glaucoma medications by some patients); other complications were uncommon. IOP of more than 21 mm Hg was associated with both increased AC cells and elevated flare (both P < or = .001) and with macular involvement (P = .009). Inflammation seemed to be more severe among patients in Brazil than among those at other sites. CONCLUSIONS: There is substantial variation between patients in the severity of intraocular inflammation associated with ocular toxoplasmosis, attributable to multiple host- and disease-related factors. Results suggest that disease characteristics also vary in different areas of the world. Elevated IOP at initial examination reflects the severity of inflammation.

Toxoplasmosis.

PURPOSE OF REVIEW: The purpose of this review is to update the latest information on a long-recognized infectious disease, ocular toxoplasmosis. RECENT FINDINGS: Topics include how the disease is acquired, how to make a clinical diagnosis, and what laboratory tests to order when the diagnosis is not clear. New insights into the genetic make-up of the parasite and new treatment alternatives are discussed. SUMMARY: Recent findings are updated on the epidemiologic status of the disease, methods for correct diagnosis of unusual presentations, and alternative therapies.

Lesión destructiva de la línea media: granulomatosis de Wegener / Medline destructive lesion: Wegener’s granulomatosis

Un paciente de 38 años de edad fue derivado con diagnóstico de leishmaniasis mucocutánea por un proceso destructivo de la nariz y los senos paranasales, con severo compromiso oftalmológico. Este trabajo discute los diagnósticos diferenciales de las lesiones destructivas de la línea media. El estudio de estas lesiones es dificultoso, pero debe efectuarse con una aproximación sistemática y múltiples técnicas diagnósticas. El diagnóstico final fue de Granulomatosisde Wegener.

Diagnostic approaches to severe, atypical toxoplasmosis mimicking acute retinal necrosis.

PURPOSE: To describe the means of diagnosis and clinical features of atypical toxoplasmic chorioretinitis mimicking acute retinal necrosis. DESIGN: Observational case series. PARTICIPANTS: Twenty-two patients (25 eyes) with widespread chorioretinitis resulting from toxoplasmosis examined between 1990 and 2001. TESTING: Patients were diagnosed by various techniques, including polymerase chain reaction (PCR) of aqueous and vitreous, serum and intraocular antibody determination, culture of intraocular fluid, retinal biopsy, histopathologic examination, therapeutic trial of antibiotics active against toxoplasmosis, or a combination thereof. MAIN OUTCOME MEASURES: The primary outcome measure was diagnosis of disseminated toxoplasmic chorioretinitis by any combination of tests or by empiric use of specific antibiotics. The secondary outcome measure was visual and anatomic outcome of treatment. RESULTS: Mean age was 53.5 years (range, 19-77 years), with a median of 59.5 years. There were 9 women and 13 men. Six patients were infected with HIV, and 3 patients, 1 with HIV, had bilateral disease. Mean initial vision was 20/110 (median, 20/400; range, 20/20 to no light perception [NLP]). Sixteen patients (73%) had received oral or injectable corticosteroids and 11 (50%) had received antiviral therapy before the diagnosis of toxoplasmosis. Diagnosis was made solely by clinical response to antitoxoplasmosis medications in 4 patients. Sixteen patients were diagnosed based on evaluation of intraocular fluids and tissue by antibody determinations, culture, PCR, histopathologic examination, or a combination thereof. Visual acuity improved after treatment in 7 of 25 eyes (28%). Mean final visual acuity was 20/156 (median, 20/2500; range, 20/30 to NLP). Anatomically, 18 of 23 eyes with follow-up had healed or improved chorioretinitis. Retinitis was progressive in 1 eye, 2 eyes were enucleated, and 2 were phthisical. CONCLUSIONS: Diagnosis of atypical toxoplasmic chorioretinitis that mimics viral retinitis can be accomplished by several means. Prompt diagnosis may help avoid poor visual and anatomic outcomes after prolonged initial treatment with oral prednisone or antiviral medications.