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Aim: To describe the different retinal morphological characteristics that can present on optical coherence topography (OCT) in a spectrum of uveitic diseases., Methods: We reviewed the literature and our own OCT image archive for characteristic features that may be suggestive of a particular disease process., Results: OCT demonstrates a variety of characteristic morphological changes, some that may point towards a specific disease process. We describe the various forms of macular oedema found in uveitis as well as OCT features typically found in multifocal choroiditis, serpiginous chorioretinitis, toxoplasma chorioretinitis, Vogt-Koyanagi-Harada, sympathetic ophthalmia and the vitreomacular traction syndrome., Conclusion: Ophthalmologists should be aware of the variety of retinal morphological characteristics that can present on OCT in uveitic disease. Recognition may aid in the diagnostic process, which is complementary to conventional fundal photography and fluorescein angiography. This can facilitate earlier diagnosis and, more importantly, the initiation of specific treatment.

Purpose: To determine the diagnostic yield of tests commonly used for vitreous fluid analysis in eyes with suspected intraocular infection or malignancy., Design: Noncomparative interventional case series., Participants: Forty-four consecutive patients (45 eyes) treated from 1998 through 2006 with posterior segment inflammation who underwent pars plana vitrectomy for diagnostic purposes., Intervention: Vitreous specimens obtained via pars plana vitrectomy were analyzed by microbiologic culture, cytologic analysis, and flow cytometry., Main Outcome Measures: Diagnostic yield and sensitivity of each test performed on vitreous specimens and visual outcomes of eyes that underwent diagnostic vitrectomy (DVx)., Results: Preoperative diagnoses were infection in 15 eyes and malignancy in 30 eyes. Overall, vitreous analysis identified a specific cause in 9 (20%) of 45 eyes. The overall sensitivity of DVx was 63.6%. The sensitivities of individual tests were: culture, 50%; cytologic analysis, 66.7%; and flow cytometry, 83.3%. The yields of diagnostic tests were: culture, 5.7%; cytologic analysis, 14.3%; and flow cytometry, 20.6%. Final diagnoses were infection in 6 eyes, malignancy in 9 eyes, and idiopathic in 30 eyes. Mean visual acuity improved significantly in the first 6 months after DVx. Visual acuity improved in 60% of eyes, with 37.8% of eyes improving by 3 lines or more., Conclusions: Analysis of vitreous fluid by widely available tests is useful in identifying intraocular infection or malignancy. Most patients experienced a substantial improvement in vision.

Purpose: Several studies have shown the capacity of interferon-alpha (IFN-alpha) to control ocular Behçet disease. The authors aimed to determine whether IFN-alpha was effective in treating patients with severe, refractory sight-threatening intraocular inflammation (uveitis) from a wider range of causes, including Behçet disease., Design: Prospective, interventional case series., Methods: Twelve patients with sight-threatening uveitis that failed to respond to one or more immunosuppressive regimens were enrolled to this study. Recombinant human IFN-alpha-2b was administered subcutaneously daily, and the dose was adjusted according to the clinical response. Main outcome measures were visual acuity, clinical activity of uveitis (including binocular indirect ophthalmoscopy [BIO] score and presence or absence of macular edema), and adverse effects of the treatment., Results: The mean observation period was 11 months (range, one to 29 months). A positive clinical response was observed in 83% of patients. Median visual acuity improved from 0.54 to 0.2 (logarithm of the minimum angle of resolution units; P < .001) and median BIO score decreased from 1.0 to 0.5 (P < .05) within one month of treatment. Macular edema, if present, resolved in all patients within days of treatment. The main adverse events were tiredness, lymphopenia, flu-like symptoms, and transient increase of liver enzymes. Weight loss occurred in four patients. Four patients experienced depression, one of them attempting suicide. Three patients experienced typical features of IFN-alpha-associated retinopathy, which resolved on reducing the dose., Conclusions: IFN-alpha seems to have significant potential in treatment of severe, sight-threatening refractory uveitis from a variety of causes. A range of adverse events, including IFN-alpha-associated retinopathy, may occur and could limit the use of this immunomodulatory drug.

Purpose: To evaluate the demographic and clinical features of punctate inner choroidopathy (PIC)., Design: Noncomparative survey., Participants: Persons with PIC who were in contact with the PIC Society., Methods: A survey was designed and posted on the Web site of the PIC Society, and persons diagnosed with PIC were invited to participate. Seventy-seven persons with PIC completed the survey., Main Outcome Measures: Demographics, presenting symptoms, disease manifestations, treatment regimens, and ocular complications., Results: Of the 77 participants who completed the survey, 90% were women, 97% Caucasian, and 85% myopic. The median age of participants was 30 years (range, 15-55). The median duration of disease at the time of survey completion was 3 years (range, <1-14). Reported presenting symptoms of PIC included scotomata (91%), blurred vision (86%), photopsias (73%), floaters (69%), photophobia (69%), metamorphopsia (65%), and loss of peripheral vision (26%). Eighty-five percent reported unilateral scotomata initially. Thirty-two percent of participants reported waxing and waning symptoms before treatment. The majority of participants (86%) had received treatment, most commonly with systemic (60%) and/or intraocular corticosteroids (22%). Fourteen percent of participants reported treatment with at least one immunosuppressive agent at some time during their disease. Sixty-nine percent had been diagnosed with choroidal neovascularization and 56% with subretinal fibrosis in at least one eye. In 75% of respondents, the onset of choroidal neovascularization occurred less than 1 year after the presenting symptoms of PIC, and in 79%, the onset of subretinal fibrosis occurred less than 1 year after the presenting symptoms., Conclusions: In this survey of 77 persons with PIC, the majority of respondents were young, myopic, Caucasian women, who experienced unilateral scotoma and blurred vision as initial symptoms. The ocular complications choroidal neovascularization and subretinal fibrosis were present in the majority of participants in at least one eye and typically occurred within the first year of initial symptoms.

Purpose: To report a case of rare posterior eye manifestation of Crohn's disease preceding recurrence of inflammatory bowel disease., Methods: Case report with ophthalmoscopic findings, fluorescein/indocyanin green angiograms, automated perimetry and multifocal-ERG., Results: The perimetry revealed absolute and relative scotomas corresponding to multifocal inflammatory lesions in the retina and choroid, reduced a/b amplitudes in multifocal-ERG and hypofluorescent dots in angiography. Under oral prednisolone visual defects, ophthalmoscopic and angiographic findings resolved, while a/b amplitudes remained mildly reduced. The ocular changes occurred without systemic hypertension and were followed by a new episode of intestinal symptoms., Conclusion: Multifocal inflammatory lesions in the retina and choroid in patients with Crohn's disease may occur and may precede a recurrent intestinal episode. Crohn's patients should be carefully followed up in collaboration with internal medicine specialists.

Sight-threatening intraocular inflammation affecting the posterior segment of the eye may be predominantly located in the peripheral retina and vitreous (intermediate uveitis) or postequatorially where it manifests as inflammation of the retina, retinal vessels and/or optic nerve with cellular infiltration of the choroid and retina and edema particularly at the macula. Involvement of the macula is the main cause for visual loss. Experimental models of posterior uveitis have revealed much concerning the mechanisms of inflammatory cell damage to the retina, implicating CD4 T cells, effector macrophages and pro-inflammatory cytokines. In particular, transgenic and gene deletion models of inflammation have allowed an understanding of how immune privilege in the posterior segment of the eye is disrupted. Importantly, this has led to the development of new treatments with novel immunosuppressants and 'biologics' and the promise of cell-based therapies which may allow customized therapies tailored to the individual's inflammatory profile.

Purpose: To perform a prospective pilot study to evaluate interferon alfa-2a (IFN alfa-2a) for the treatment of refractory cystoid macular edema (CME) in endogenous uveitis., Methods: IFN alfa-2a was administered at an initial dose of 3 or 6 million IU (depending on body weight) per day subcutaneously. Afterwards IFN alfa-2a was tapered slowly over 6 months and finally discontinued. If CME relapsed IFN alfa-2a was reinstituted and tapered slowly again to evaluate the lowest maintenance dose to keep remission., Results: A total of 15 eyes of 8 patients with refractory CME due to intermediate or posterior uveitis were included. Ineffective pretreatment consisted of systemic steroids and acetazolamide (all patients) and at least one additional immunosuppressant (6 patients). Six of 8 patients (11 eyes) responded well to IFN alfa-2a and CME resolved completely during 6 months treatment. One patient was lost to follow-up after IFN alfa-2a was stopped. In 1 patient (1 eye) even 19 months after cessation of IFN alfa-2a no recurrence of CME occurred. In 4 patients (8 eyes) IFN alfa-2a had to be reinstituted because CME relapsed. All 4 patients responded again. During a mean follow-up period of 16.4 months since restart of therapy we succeeded in all 4 patients to taper IFN alfa-2a to maintenance doses between 1.5 million IU every second and every sixth day without a recurrence of CME in any of the 8 eyes., Conclusion: IFN alfa-2a can be a treatment option for patients with otherwise treatment resistant uveitic CME.

Background: After exclusion of a masquerade syndrome, uveitis may be associated with infection, systemic diseases, specific ocular diseases, or may be drug-induced. In order to improve diagnostic strategy in uveitis, we performed a comparative study to assess the importance of internist and ophtalmologist's collaboration and we proposed a well-adapted diagnostic procedure., Material and Methods: A comparative study was performed in a tertiary centre. The population was divided in two groups. The first one was retrospective and consisted of patients treated at the Ophthalmology department of Croix Rousse Hospital from 1991 to 2002 without internist's collaboration. The second one was prospective and consisted of patients referred in the same centre in 2003 and 2004 with intervention of an internist. Patients of less than 18 years of age with pre-existent diagnosis, specific ocular diseases, toxoplasmosis infection, or with human immunodeficiency virus infection were excluded., Results: Sixty-six patients were included. Anterior uveitis was the most frequent form (25 cases), followed by panuveitis (20 cases), posterior uveitis (14) and intermediate uveitis (7). The most frequent cause of uveitis was systemic disease (19 cases) followed by infection (7 cases) and neurological entities (4 cases). The rate of diagnosis was 30.3% without internist's intervention and 60.6% when patients were referred to an internist (P=0,01). The internist intervention was contributive in 75% of diagnoses., Conclusions: The internist intervention significantly enhances the rate of etiological diagnoses in uveitis. These results should further strengthen the internist/ophthalmologist collaboration for patients with uveitis.

Purpose: To report on two patients who developed rapid progression of asymmetric diabetic retinopathy (DRP) in eyes affected by posterior uveitis in contrast to their fellow eyes not affected by uveitis., Design: Observational case report., Methods: Two patients with diabetes mellitus (DM) and unilateral uveitis underwent repeated ophthalmologic examinations and fluorescein angiography., Results: Two patients with DM and unilateral posterior uveitis developed proliferative DRP in eyes with previous uveitis within 3 months after the uveitis subsided. In contrast, the retinal findings of nonuveitic eyes remained unchanged on follow-up of several years., Conclusions: Since the pathogenesis of intraocular inflammation and diabetic retinopathy acts through similar biochemical mediators and pathways, it is feasible that posterior uveitis accelerates the progression of diabetic retinopathy. Our results support this hypothesis and point out a risk for rapid retinopathy development in eyes affected with posterior uveitis.

Purpose: Analysis of non-uveitic entities that were referred as posterior uveitis to a uveitis referral center., Methods: We came across and analyzed 32 cases of non-uveitic entities (1.12%) that had been misdiagnosed and referred as posterior uveitis out of 2846 outpatient cases of posterior uveitis registered from 1990 to June 2002. The final diagnosis was achieved by history, slit-lamp biomicroscopy, meticulous fundus evaluation, imaging, and ancillary studies., Results: The finally diagnosed 32 non-uveitic entities that mimicked posterior uveitis were multiple leak central serous retinopathy (n = 8; 25%), choroidal neovascular membrane (n = 6; 18.75%), typical central serous retinopathy (n = 4; 12.5%), dry form of age-related macular degeneration (n = 3; 9.3%), retinitis pigmentosa (n = 3; 9.3%), heredomacular degeneration (n = 3; 9.3%), myelinated nerve fibers (n = 2; 6.2%), malignant melanoma of the choroid (n = 1; 3.1%), old spontaneously reattached retinal detachment (n = 1; 3.1%), and subhyaloid hemorrhage (n = 1; 3.1%)., Conclusion: The purpose of the study was to underline the fact that there are some non-uveitic entities that mimic posterior uveitis and that an ophthalmologist can be deceived. The study highlights the problems diagnosing posterior uveitis and presents an algorithm of diagnostic approach to avoid misdiagnosis.

Purpose: To present and evaluate research criteria for the diagnosis of birdshot chorioretinopathy., Design: Consensus statement., Methods: Investigators who attended an international conference on birdshot chorioretinopathy drafted diagnostic criteria, which were refined after comment by conference attendees. Sensitivity and specificity of the criteria were evaluated by review of 82 previously diagnosed cases and evaluation of 80 consecutive patients with other forms of uveitis., Results: Criteria, including presence of "birdshot lesions" and mild intraocular inflammation in both eyes, are based on clinical findings in the absence of systemic disease; HLA-A29 is supportive of diagnosis, but not required. Diagnostic criteria were found to be 100% specific and 97.5% sensitive. Positive predictive value was 100%; negative predictive value was 97.6%., Conclusions: Standardized criteria for the diagnosis of birdshot chorioretinopathy will facilitate clinical research and the comparison of results from studies performed at different institutions by ensuring a homogeneous population of study patients.

Purpose: To describe baseline clinical characteristics of a cohort of 80 patients with birdshot chorioretinopathy in anticipation of a longitudinal study, and to identify relationships between visual acuity, symptoms, and ophthalmic findings., Design: Single-center cross-sectional study., Methods: A standardized examination was performed in the same order on a single day for each patient. A grading system for birdshot lesions was established prospectively to evaluate the following lesion characteristics: quantity, distribution, morphology, and pigmentation. Relationships between clinical features of disease were sought in multivariate analyses that adjusted for age, duration of uveitis, and treatment., Results: Mean age at baseline examination was 55.6 years. Median best-corrected visual acuity (BCVA) was 0.8 (range, counting fingers to 1.2). There were no relationships between BCVA and any birdshot lesion characteristic. The most common cause of BCVA < or =0.4 was macular edema. Visual symptoms were present in 78 patients (97.5%), including 17 (94.4%) of 18 patients with BCVA > or =1.0 in both eyes. Blurred vision was associated with decreased BCVA (P = .02) and macular edema (P = .022). Increased lesion pigmentation was associated with complaints of blurred vision (P = .030), vibrating vision (P = .011), and nyctalopia (P = .056)., Conclusions: Symptoms are common in patients with birdshot chorioretinopathy, even among those with good BCVA. Lesion pigmentation may be a marker of decreased visual function that is not reflected in central visual acuity. These findings highlight the limitation of using visual acuity measurements for monitoring patients with birdshot chorioretinopathy and as an outcome measure for studies of this disease.

Background: To describe the prevalence, clinical features, and causes of vision loss among patients with ocular toxocariasis seen at a uveitis referral center., Methods: A review was completed of the charts of patients with ocular toxocariasis who were examined between 1977 and 1996 at the Francis I. Proctor Foundation of the University of California at San Francisco. The prevalence of ocular toxocariasis among all uveitis patients seen at the center was determined. Demographic features, symptoms, and signs in all patients were evaluated., Results: Ocular toxocariasis occurred in 22 (1.0%) of 2,185 uveitis patients. The mean patient age was 16.5 years. Inflammation was usually unilateral (90.9%). Toxocara uveitis presented as a granuloma in the peripheral retina in 50% of cases, as a granuloma in the macula in 25% of cases, and as a moderate to severe vitreous inflammation mimicking endophthalmitis in 25% cases. The primary causes of vision loss were vitritis (52.6%), cystoid macular edema (47.4%), and traction retinal detachment (36.8%)., Conclusions: Ocular toxocariasis is an uncommon cause of uveitis that mainly affects younger patients. Inflammation is typically unilateral and presents as either a granuloma in the peripheral or posterior retina or a moderate to severe vitreous inflammation mimicking endophthalmitis.

Toxoplasmosis is the most common cause of posterior uveitis in immunocompetent subjects. The infection can be congenital or acquired. Ocular symptoms are variable according to the age of the subject. For instance, young children present with reduced visual acuity, strabismus, nystagmus, and leucocoria, while teenagers and adults complain of decreased vision, floaters, photophobia, pain, and hyperemia. Toxoplasmic retinochoroiditis typically affects the posterior pole, and the lesions can be solitary, multiple or satellite to a pigmented retinal scar. Active lesions present as grey-white focus of retinal necrosis with adjacent choroiditis, vasculitis, hemorrhage and vitreitis. Cicatrization occurs from the periphery towards the center, with variable pigmentary hyperplasia. Anterior uveitis is a common finding, with mutton-fat keratic precipitates, fibrine, cells and flare, iris nodules and posterior synechiae. Atypical presentations include punctate outer retinitis, neuroretinitis, papillitis, pseudo-multiple retinochoroiditis, intraocular inflammation without retinochoroiditis, unilateral pigmentary retinopathy, Fuchs'-like anterior uveitis, scleritis and multifocal or diffuse necrotizing retinitis. The laboratory diagnosis of toxoplasmosis is based on detection of antibodies and T. gondii DNA using polymerase chain reaction (PCR). Toxoplasmosis therapy includes specific medication and corticosteroids. There are several regimens, with different drug combinations. Medications include pirimetamine, sulfadiazine, clindamycin, trimethoprime-sulphamethoxazol, spiramycin, azithromycin, atovaquone, tetracycline and minocycline. The prognosis of ocular toxoplasmosis is usually good in immunocompetent individuals, as long as the central macula is not directly involved.

Purpose: We analyzed the epidemiologic data and vision-threatening complications in different forms of childhood uveitis., Methods: This retrospective study included 187 consecutive patients with onset of uveitis before the age of 16 years classified as anterior (AU), intermediate (IU), posterior (PU), and panuveitis (PanU). We analyzed the epidemiologic data as well as visual acuity, uveitic complications and the conservative and surgical therapy., Results: Associated disease was observed in 85 of 187 patients. The most common complications in AU patients were cataract, posterior synechiae, band keratopathy and CME. IU was accompanied by dense vitreous opacities, cataract and CME. Macular scars were the most frequent cause for visual loss in PU. PanU was complicated by dense vitreous opacities, cataract, retinal detachment, CME and phthisis bulbi., Conclusions: Childhood uveitis is frequently associated with systemic immune-mediated diseases. The diverse uveitis types have different but typical complications.

Purpose: To assess the long-term effect of acetazolamide treatment on patients with cystoid macular edema (CME) in the course of intermediate or posterior chronic uveitis and to define those patients who may particularly benefit from the drug., Methods: Fifty-two eyes (45 patients) with chronic uveitic CME were treated with acetazolamide at an initial dosage of 500 mg/d. The effect of treatment was assessed by fluorescein angiography, ophthalmoscopy, visual acuity, and Amsler testing. Therapy was withdrawn when CME did not improve at 3 weeks. In cases with CME improvement, the dosage was gradually tapered., Results: The mean follow-up was 3.1 years (minimum, 1.5 years). Two subgroups were identified: group 1, quiescence of uveitis with acetazolamide as the single therapeutic agent (33 eyes); and group 2, chronically active uveitis requiring additional systemic antiinflammatory drugs (19 eyes). In both groups, visual acuity improvement was statistically significant (group 1, P = 0.012; group 2, P = 0.025). In 12 patients with a stable visual acuity gain, the medication dose could be tapered off completely without any recurrent edema shown by fluorescein angiography after a minimum follow-up of 1 year. Sixteen patients required a maintenance dosage, ranging from 125 to 500 mg daily. No major adverse effects of the medication were observed., Conclusions: During long-term follow-up, low-dose acetazolamide can be a useful therapeutic option for chronic CME in uveitis. The effect was better in patients with quiescence of uveitis than in those with chronically active uveitis. Permanent therapy is not imperative in every case.

Purpose: To report the value of endoretinal biopsy in establishing the diagnosis of severe posterior uveitis., Methods: A clinicopathologic report of three patients with severe posterior uveitis unresponsive to corticosteroids and with a negative systemic workup. These patients subsequently underwent vitrectomy and endoretinal biopsy., Results: Endoretinal biopsy provided an accurate diagnosis in the three cases, leading to appropriate treatment. Histopathologic examination of the three retinal samples confirmed the diagnosis of sarcoidosis, tuberculosis, and lymphoma. Targeted treatment improved the ocular conditions in all three cases., Conclusions: In selected cases of severe bilateral vision-threatening uveitis, retinal biopsy may be indicated as a last resort to confirm a suspected diagnosis of infectious disease or malignancy.

Objective: To report indocyanine green (ICG) angiography anomalies in ocular syphilis., Design: A noncomparative, interventional case series., Participants: Eight patients (16 eyes) affected by ocular syphilis., Methods: All patients presenting with a diagnosis of active ocular syphilis between January 1994 and December 2001 were evaluated by a standard fluorescein and ICG angiography protocol. ICG angiography was repeated after completion of systemic antitreponemal and antiinflammatory treatment., Results: ICG angiography anomalies were detected in 12 (75%) of 16 eyes. Two types of anomalies were observed: late-phase scattered hyperfluorescent spots (11 eyes) and persistent staining of retinal vessels (1 eye). Of these 12 eyes, 4 had no alteration by concomitant fluorescein angiography. When ICG angiography was repeated (5 +/- 1 weeks after the beginning of treatment), ICG angiography anomalies disappeared., Conclusions: ICG angiography may be a valuable tool in the assessment of patients with active ocular syphilis. Retinal and choroidal vascular anomalies can be determined that would otherwise go undetected by funduscopy and/or fluorescein angiography. It can also be useful in monitoring antitreponemal therapy.

26 patients with active posterior uveitis were studied by methodologies for the confirmation of leptospirosis. ELISA-IgG was positive in 10 patients and MAT in 21. Leptospiras were observed in blood and urine. The results suggest that posterior uveitis may be caused by pathogenic leptospiras.

Purpose of Review: The goal of this review is to describe the more commonly used imaging techniques and their use to identify causes of vision loss, extent of disease, and distinctive patterns associated with various causes of posterior uveitis., Recent Findings: Distinctive patterns seen with new imaging techniques and applications are being described. Ophthalmic CT, fluorescein angiography, indocyanine green angiography, and others are demonstrating inflammation and pathology in posterior uveitis. As our experience grows with these modalities, they are being used increasingly in the diagnosis and management of patients with posterior uveitis., Summary: This review familiarizes the ophthalmologist with imaging in patients with inflammatory disorders of the retina and choroid. These modalities can help with the diagnosis, treatment, and monitoring of patients with uveitis.

A 48-year-old male was admitted to our hospital because of abnormal pulmonary shadows and a decrease in visual acuity. He had a history of tuberculosis 20 years ago. The chest roentgenogram showed multiple pulmonary nodules throughout both lung fields. No definitive diagnosis was established either by brushing cytology or biopsy through bronchoscopy or percutaneous needle biopsy. Pathological examination of open lung biopsy specimen revealed that extensive, hyalinized lamellar collagen bundles arranged in whorls, parallel arrays. Plasma cells and lymphocytes were found between the collagen bands and germinal centers were seen at the periphery of the lesion. A definitive diagnosis of pulmonary hyalinizing granuloma was made on the basis of these histopathological findings. Although there is no established treatment for pulmonary hyalinizing granuloma, during 1 month of follow-up, posterior uveitis mildly resolved with glucocorticoid treatment and there had been a slight increase in visual acuity.

Objectives: To assess the clinical value of retinal or choroidal biopsy in establishing a definitive diagnosis directing subsequent management and to assess the risk involved in such surgery., Design: Retrospective noncomparative interventional case series., Participants: Thirteen patients with uveitis of suspected malignant or infectious origin. One patient had 2 consecutive biopsies on the same eye., Methods: A retrospective review of a 4-year period was undertaken of all those cases in which the biopsy was taken for diagnostic purposes at a single institution., Main Outcome Measures: Ability of biopsy to establish a pathologic diagnosis and guide treatment and surgical complications., Results: A transscleral approach was used in 4 and a pars plana vitrectomy approach in 10 of the biopsies. The only operative complication was a retinal break in 1 case. Postoperative events that might be related to the surgery included 1 localized retinal detachment, 2 cataracts, and 1 phthisical eye. The pathologic diagnosis differed from the initial clinical diagnosis in 5 of the 13 cases. The biopsy result directed specific appropriate treatment in 7 of the 13 cases. In the 6 remaining cases, no specific diagnosis was confirmed, but the results of the biopsy were useful in excluding malignancy and in some cases allowing a therapeutic trial., Conclusions: Retinal or choroidal biopsy is a valuable investigation that often changes clinical management when carried out for specific indications in patients with uveitis of suspected malignant or infectious origin. Although it is likely to remain an investigation of last resort, it should be considered at an earlier stage when there is diagnostic uncertainty if severe visual loss is to be prevented.

Purpose: To determine the role of indocyanine green angiography (ICG) in handling chorioretinal inflammatory disorders., Method: We present several posterior uveitis whose diagnosis and therapeutic approach was facilitated with ICG., Results: ICG is useful in the differential diagnosis of <>. In serpiginous choroiditis, the edges and the progression of the inflammatory areas are clearly delineated. In infectious uveitis such as toxoplasmosis, ICG determines the presence or absence of active lesions. There are granulomatous diseases such as sarcoidosis where some areas of choroidal inflammation that cannot be detected with other methods are shown by ICG. Moreover, ICG can detect areas of subretinal neovascularization in some choroidal pathologies such as polypoidal choroidal vasculopathy., Conclusions: ICG appears to be a useful and complementary tool in the clinical interpretation of posterior uveitis.

The authors discuss methods of improvement of the etiologic diagnosis of the toxoplasma and toxocara associated posterior uveititis, by examining specific antibodies in the aqueous humor. Paired samples of aqueous humor and serum were analyzed in 23 patients. Chorioretinitis of infectious etiology was suspected in 15 patients, 3 patients were treated for panuveitis, 4 patients for intermediate uveitis, and in 1 patient the sample was obtained to rule out malignant melanoma. The local ocular antibody production was calculated according to Desmonts. Toxoplasma etiology was confirmed by local antibody production analysis in 1 patient, toxocara etiology in 5 patients. In 20 patients in the control group local specific antibody production was not demonstrated.

Unlabelled: Two young boys had a severe uveitis which was difficult to treat. They both developed psoriasis located in the pubic area almost 1 year and several years later respectively. One boy also developed arthritis of an intermittent character. Both boys were ANA and HLA-B27 negative., Conclusion: in children, the possible association between uveitis and psoriasis with or without arthritis has to be kept in mind.

Objective: Aqueous humor analysis can be performed in severe atypical forms of posterior uveitis unresponsive to conventional treatment to exclude a viral infection., Design: Noncomparative interventional case series., Participants: Thirty-seven immunocompetent patients seen with corticosteroid-resistant forms of posterior uveitis underwent extensive evaluation, including anterior chamber paracentesis, to rule out a nonnecrotizing viral retinopathy., Intervention: Aqueous fluid samples were prospectively obtained. Polymerase chain reaction (PCR) and serologic evaluation of intraocular antibody production against herpesviruses were performed by molecular techniques and enzyme-linked immunosorbent assay., Main Outcome Measures: Polymerase chain reaction and local antibody production for herpes simplex virus types 1 and 2, varicella-zoster virus, cytomegalovirus, and Epstein-Barr virus were determined on aqueous fluid samples., Results: Viral infection was confirmed in 5 cases (13.5%). Clinical presentation included birdshot-like retinochoroidopathy, occlusive bilateral vasculitis, and cystoid macular edema. An antiviral regimen was initiated in all cases. Inflammation was stabilized, and steroid dosage could be significantly reduced., Conclusions: Identification of a viral agent during severe posterior uveitis can dramatically change therapeutic management.

Background: Although the tubulointerstitial nephritis and uveitis (TINU) syndrome has been reported, there are only a few reports in the ophthalmological literature. The ocular findings usually appear later than the renal findings, and a renal biopsy is required for a definitive diagnosis., Cases: Case 1 was a 15-year-old Japanese girl who was a carrier of the human T lymphotropic virus, type 1 (HTLV-1). She had tubulointerstitial nephritis, anterior uveitis, posterior retinal edema in the right eye, and peripheral vasculitis OU. Case 2 was a 56-year-old Japanese woman who was also a carrier of HTLV-1. Previously she had been diagnosed with Graves' disease, anterior uveitis, and vitreous opacity. Several years later tubulointerstitial nephritis was diagnosed., Observations: In both patients interstitial nephritis was diagnosed and systemic steroid therapy was effective., Conclusions: It is difficult for ophthalmologists to diagnose the TINU syndrome because the renal biopsy is performed by a pediatrician or an internist. This may be why ophthalmologic descriptions are rare in cases of TINU syndrome, even though the ocular findings reported in the literature are characteristic. This is the first case report of TINU syndrome in patients with HTLV-1. Ophthalmologists should pay more attention to the uveitis in the TINU syndrome.

Purpose: Pediatric uveitis is rare and has been reported to cause increased rates of visual loss compared with adult patients. The reasons for this are unclear. Only one study has been population-based, so the effect of referral bias is not known. We examined the pattern of disease in primary and referral centers to establish the unique characteristics of uveitis in children., Design: Case control study., Methods: Retrospective, multicenter, observational study of uveitis starting before the age of 20 years. Two hundred forty-nine patients were recruited from three primary and two referral ophthalmic units. Age-related differences in types of uveitis and systemic disease between hospitals were characterized, as were associations with visual loss., Results: The incidence of uveitis in district hospitals at less than 16 years of age was 4.9/100,000: the most frequent diagnosis was idiopathic uveitis (78%). In referral cohorts the most frequent diagnosis was juvenile idiopathic arthritis-associated uveitis (67%). Other systemic diseases were rare. The most frequent type of uveitis at 0 to 7 years of age was chronic anterior uveitis, posterior uveitis in 8- to 15-year-olds, and acute anterior uveitis in 16- to 19-year-olds. Visual loss (any eye < 6/12) occurred in 17% and was not associated with age, sex, or hospital cohort. It was most frequent in posterior uveitis (25%). Treatment variables were independent predictors of visual loss: systemic treatment 2.2 (1.1- 4.6), surgical intervention 8.2 (3.8-17.6)., Conclusions: Idiopathic uveitis was three times more common in district hospitals. Visual loss was similar to adult uveitis in this study. The increased frequency of severe chronic anterior uveitis in children aged 0 to 7 years and posterior uveitis in older children aged 8 to 15 years accounts for the rate of visual loss seen in previous studies.

Polymerase chain reaction (PCR) is a highly sensitive and specific method for detection and quantification of specific nucleic acids from a clinical sample. With its use, genetic, infectious, neoplastic, and autoimmune diseases can be diagnosed and managed with a high level of sensitivity, accuracy, and rapidity. This technique exactly reproduces unlimited copies of DNA, even if only a small amount are present initially. PCR assays can detect presence of fastidious and slow-growing microorganisms, such as chlamydia, mycoplasmas, mycobacterias, and viruses directly from clinical specimens and also can detect antimicrobial resistance. The value of viral load measurement by nucleic acid amplification in the management of patients with HIV infection or hepatitis C has also been well established. From the point of view of a clinician, the applications of PCR are focused mainly in the amplification and detection of diagnostic DNA segments from the genomes of both pathogens and patients.

Objective: To validate a real-time polymerase chain reaction (PCR) assay allowing rapid and sensitive detection and quantitation of 4 common infectious posterior uveitis pathogens., Methods: A real-time PCR assay using previously validated primer sets for cytomegalovirus, herpes simplex virus, varicella-zoster virus, and Toxoplasma gondii was developed. A standard curve for quantitation of pathogen load was generated for each pathogen using SYBR Green I fluorescence detection. Ocular samples from patients with posterior uveitis and from negative control samples were assayed and compared with standards to identify pathogens and quantify infectious load., Results: Sensitivity for detection of purified pathogen DNA by PCR was not reduced by application of the real-time method. Standard curves for the quantitation of pathogen loads showed sensitivity to fewer than 10 organisms for all pathogens. The technique was applied to 2 clinical problems. First, sensitivities of existing monoplex and multiplex PCR were compared by real-time PCR. No significant difference in sensitivity was observed between multiplex and monoplex techniques. Second, pathogen loads of vitreous specimens from patients previously diagnosed as having infectious posterior uveitis were calculated. Pathogen loads were found to be generally higher for patients with disease caused by varicella-zoster virus than those caused by cytomegalovirus or herpes simplex virus., Conclusions: Real-time PCR may be applied to infectious agents responsible for posterior uveitis. This technique will likely prove useful for the diagnosis of posterior uveitis as well as the linkage of pathogen to disease., Clinical Relevance: Real-time PCR provides a rapid technique for quantitatively evaluating ocular samples for the presence of infectious pathogens.

Birdshot retinochoroidopathy (BSR) is a bilateral posterior uveitis. A putative organ-specific autoimmune disease, it is strongly associated with the HLA-A29 allele, and understanding the immunopathogenesis of BSR is of great interest. The clinical features include minimal anterior uveitis, vitritis, retinal vasculitis, cystoid macular edema, and distinctive hypopigmented choroidal lesions. Findings on electrophysiology studies and angiography have implications for understanding the pathophysiology of the disease, and may be useful for following the course of BSR and the response to therapy in individual patients. The decision to initiate therapy can be difficult, but corticosteroids and immunosuppressive agents are often used.

Purpose: To provide long-term follow-up information on Eales' patients., Methods: Eales' patients, who had been examined at varying periods between the years 1970 and 1991 with a minimum five-year follow-up, were included in the study., Results: A total of 130 patients were followed up for a minimum of five and a maximum of 26.5 years. The retinal lesions found during the first examination included vascular sheathing, disc and/or retinal neovascularization, vitreous hemorrhage, branch retinal vein occlusion, retinitis proliferans, and retinal detachment. Visual acuity improved in 37 (20%) of the 185 treated eyes, was maintained in 79 (43%), and worsened in 69 (37%). The complications were tractional detachment, cataract, rubeosis iridis, neovascular glaucoma, and phthisis bulbi., Conclusion: The most important elements in dealing with Eales' disease are periodic follow-up, a good and adequate laser treatment, pars plana vitrectomy combined with procedures for nonclearing vitreous hemorrhage, and retinal vasoproliferative changes to stabilize the retinal lesions and maintain functional levels of vision.

Background: The severity of disease and the side-effects of long-term corticosteroid treatment support the rationale for other immunosuppressive drugs in the management of uveitis. Mycophenolate mofetil (MMF) is a selective inhibitor of ionosine monophosphate dehydrogenase and exerts its major effects by modulation of the function of T and B lymphocytes. This study was undertaken to evaluate the clinical effect of MMF in the control of intermediate and posterior uveitis., Methods: A retrospective study of 18 consecutive patients with intermediate or posterior uveitis treated with MMF was performed. Activity of intraocular inflammation was graded according to the guidelines of the international uveitis study group before and during treatment with MMF. Furthermore, the ability of MMF treatment to reduce the steroid dosage and/or substitute other immunosuppressive agents with unacceptable side-effects (cyclosporin A, tacrolimus, azathioprine) was evaluated., Results: The indication for treatment with MMF in all 18 patients (age range: 11-73 years) was either poor control of ocular inflammation by the previous immunosuppressive therapy or unacceptable side-effects of this therapy. The daily MMF dose was 2 g and therapy was combined with cyclosporin A and/or prednisolone. One patient received MMF monotherapy. Corticosteroids were discontinued in 4 patients and the steroid dose could be reduced in 14 patients following MMF therapy. Marked resolution of ocular inflammatory activity occurred in 13 patients. The most frequently observed side-effects of MMF were myalgia, fatigue, headache and gastrointestinal problems., Conclusion: MMF was effective in disease control in the majority of patients with intermediate and posterior uveitis and proved to be a useful second line immunosuppressant for refractory intraocular inflammatory disease with an acceptable profile of side-effects.

Purpose: To assess the clinical and epidemiological characteristics, diagnostic and therapeutic methods, and the final visual outcome of a group of patients with ocular sarcoidosis., Methods: Retrospective study of 18 patients diagnosed of ocular sarcoidosis between March 1989 and May 1999. In every patient the following data were obtained: age, sex, bilaterality, initial and final visual outcome, systemic and ocular manifestations, stage of chest x-ray, serum markers, gallium scan, results of biopsy, medical and surgical treatment, and complications of treatment., Results: The mean age at onset was 49.7 years (S.D. 19.9). Fourteen patients (77.8%) were female and 4 (22.2%) were male. The average follow-up time was 40.3 months (S.D. 28.7). Nine patients (50.0%) underwent a biopsy. The presence of non-caseating granulomata was observed in 7 (77.8%). Gallium scanning was positive in 16 cases (88.8%). The most frequent ocular manifestation was panuveitis (40.0%). Among the 30 eyes studied, conjunctival involvement was found in 10 (33.3%), secondary cataracts in 9 (30.0%), and secondary glaucoma in 6 (20.0%). Eleven patients (61.1%) were treated with oral corticosteroids and 10 (55,5%) with cyclosporine A. When sarcoidosis was diagnosed, 11 eyes (36.6%) had a visual acuity better than 0.6. At the end of the follow-up, the final visual outcome was better than 0.6 in 17 eyes (56.6%)., Conclusions: An adequate control of ocular inflammation may improve the prognosis in patients with ocular sarcoidosis. Posterior segment involvement (posterior uveitis, macular edema or epiretinal membrane) may be associated with a worse visual prognosis.

Background: By allowing one to detect fluorescence beyond the retinal pigment epithelium, indocyanine green angiography (ICGA) has made it possible to analyse the choroidal vessels. Our aim was to characterize choroidal vasculitis in posterior uveitis using ICGA., Methods: Charts of active posterior uveitis patients with a specific diagnosis seen in the different centers participating in the study who had undergone dual fluorescein and ICG angiography were reviewed. The type of inflammatory involvement of the choroidal circulation at entry and the treatment response on follow-up angiograms were analysed., Results: A total of 129 patients were analysed. Choroidal vasculitis could be subdivided into two main patterns: (1) primary inflammatory choriocapillaropathy and (2) stromal inflammatory vasculopathy. The first pattern consisted of hypofluorescent areas up to the late phase of angiography characteristic for choriocapillaris non-perfusion and included entities such as multiple evanescent white dot syndrome (MEWDS), acute posterior multifocal placoid pigment epitheliopathy (APMPPE), multifocal choroiditis (MC), ampiginous choroidopathy and serpiginous choroidopathy. The second pattern consisted of fuzzy indistinct appearance of vessels in the intermediate angiographic phase and diffuse choroidal hyperfluorescence in the late phase indicating inflammatory vasculopathy of larger choroidal vessels. This pattern was found in all cases of active Vogt-Koyanagi-Harada disease, ocular sarcoidosis and tuberculosis and birdshot chorioretinopathy. In Behçet's uveitis of recent onset, choriocapillaris perfusion delay and fuzzy choroidal vessels without diffuse late choroidal hyperfluorescence was found. In posterior scleritis, enlargement of vorticous veins was an additionnal ICGA sign. Stromal inflammatory vasculopathy always responded to anti-inflammatory therapy. A third group of patients with severe retinal or choroidal inflammation presented with associated secondary inflammatory choriocapillaropathy angiographically identical to the primary involvement., Conclusions: ICGA allowed the hitherto impossible characterization of inflammatory involvement of the choroidal vessels, showing either predominant inflammation of the choriocapillaris or predominant inflammation of the stromal choroidal vessels with or without secondary choriocapillaritis. ICGA will be indispensable for the correct evaluation and follow-up of posterior inflammation with suspected choroidal involvement.

The authors show that syphilis can affect the anterior and posterior ocular segment and present history, symptoms, clinical forms, laboratory tests, prognosis and treatment.

Objective: To validate a multiplex polymerase chain reaction (PCR) assay capable of simultaneously screening vitreous biopsy specimens for a panel of common pathogens in posterior uveitis., Methods: A multiplex PCR assay using novel primer sets for cytomegalovirus (CMV), herpes simplex virus (HSV), varicella zoster virus (VZV), and Toxoplasma gondii was developed. The sensitivity of the assay was determined for purified pathogen DNA. Twenty-one vitreous specimens from patients with posterior uveitis were tested by both multiplex and monoplex PCR., Results: Fewer than 10 genomes of VZV and fewer than 100 genomes of HSV, CMV, and T gondii could be detected using the new primer sets. When used in multiplex, the assay lost less than 1 log of sensitivity. Monoplex PCR detected pathogen DNA in 18 of 21 patient samples; multiplex PCR detected pathogen DNA in 15 of the 18 samples positive by monoplex PCR. None of 10 negative control samples were positive for pathogen DNA., Conclusions: Multiplex PCR has adequate sensitivity to simultaneously screen a substantial differential diagnosis for posterior uveitis in a single reaction, without loss of specificity. This assay may reduce the time and cost involved in PCR-based molecular diagnostics of infectious pathogens., Clinical Relevance: Mutiplex PCR may allow rapid diagnosis of infectious posterior uveitis.

Objective: To present the clinical and histopathologic findings in five cases of tuberculosis (TB) with various ocular manifestations., Design: Observational case series., Methods: Retrospective review of clinical findings, course, and treatment of five patients. Diagnostic techniques, including biomicroscopic, histopathologic, and molecular biologic test results, are presented., Main Outcome Measures: Visual acuity, slit-lamp biomicroscopy, indirect ophthalmoscopy, and fluorescein angiography results., Results: The ocular manifestations of TB in our patients included panophthalmitis, endophthalmitis, posterior uveitis with choroidal tubercles, keratitis, and a lid mass. Mycobacterium tuberculosis was identified in four cases in ocular specimens using acid-fast bacilli microscopy and in three cases by culture. Rapid diagnosis using polymerase chain reaction was obtained in one case. Extraocular foci of TB were identified in three cases with an intraocular infection at presentation. No patients had the human immunodeficiency virus (HIV) and none were immunocompromised. Two eyes could not be saved using antituberculous treatment because of delayed diagnosis and treatment., Conclusions: In this age of the HIV pandemic, TB is becoming more common. Because it is curable, heightened awareness and better understanding of the disease's ocular manifestations should be of concern to all ophthalmologists.

The purpose of this study was to evaluate the efficacy of combined albendazole and steroid treatment for uveitis caused by Toxocara canis in immunocompetent patients. Five patients (7 eyes) with ocular larva migrans syndrome (OLM) were used in this study. Toxocariasis was suspected based on clinical manifestations and confirmed by anti-toxocara IgG and Western blot analysis. Systemic albendazole (adults: 800 mg b.i.d.; children: 400 mg b.i.d.) was given in conjunction with steroids. Visual acuity before and after therapy, inflammatory response, side effects and toxicity were evaluated. Treatment resulted in an improved visual acuity in all patients. Mean initial Snellen visual acuity was 20/40, and mean final acuity was 20/20. There were no recurrences of uveitis throughout the observation period (average: 13.8 months; range: 3 days to 24 months). These findings suggest that albendazole, in combination with systemic steroids, is a useful regimen to treat ocular larva migrans syndrome.

Purpose: To evaluate ocular features in patients presenting with inflammation in the presence of anticardiolipin antibodies., Methods: A descriptive study of 13 patients presenting with idiopathic ocular inflammation involving anterior and posterior segment was performed. Patients were followed for a mean follow-up of 22 months (range, 1 to 125). A comprehensive report of ocular involvement, including visual symptoms, visual acuity, clinical characteristics, funduscopic and fluorangiographic features, was reported. Systemic associated symptoms were analyzed. Laboratory investigations included anticardiolipin antibody titers and isotypes, presence of other autoantibodies, and markers of immune system activation., Results: The most common ocular symptom at presentation was blurred vision (eight patients) followed by redness and pain(three patients) and visual loss(two patients). Anterior segment abnormalities, including iritis (eight patients) scleritis (two patients) and filamentary keratitis (one patient), were present in 76% of patients, whereas the most represented feature of posterior involvement was retinal vasculitis (60%) followed by vitritis (38%), retinal detachment (15%), posterior scleritis (7%), and central retinal artery occlusion (7%). All patients had abnormal titers of anticardiolipin antibodies, predominantly IgG isotype; six had markers of immune system activation., Conclusions: Although posterior pole disease is more commonly associated with anticardiolipin antibodies, the anterior segment can also be involved with a wide spectrum of features. Scleritis has never been previously described as associated with anticardiolipin antibodies. Systemic symptoms are frequently present in association with ocular disease.

The paper presents a clinical case of Acute Posterior Multifocal Placoid Pigment Epitheliopathy and the specific diagnosis problems of this case. Being a less frequent disease the common aspects are emphasized as well as the latest concepts about this syndrome.

Purpose: By providing information on choroidal lesions, indocyanine green (ICG) angiography is complementary to fluorescein angiography in the workup of posterior uveitis. The aim here was to illustrate practically the suitability of performing dual fluorescein/ICG angiograms in a demonstrative case of recurrent presumed posterior sarcoid uveitis., Methods: Sequential dual fluorescein/ICG angiograms were performed for the workup and follow-up of a case of recurrent presumed sarcoid uveitis., Results: Dual fluorescein and ICG angiograms performed during the first recrudescence of inflammation showed mainly retinal involvement with cystoid macular edema, diffuse posterior pole retinal hyperfluorescence, and papillitis, whereas the second recurrence after tapering oral corticosteroid therapy showed new choroidal lesions of the posterior pole in the absence of significant retinal involvement., Conclusions: Dual fluorescein/ICG angiography clearly allowed the differentiation of two different inflammatory events, one involving the retina and the other involving the choroid, at the origin of similar clinical manifestations.

Background: Treatment of children with juvenile uveitis requires adequate control of inflammation while minimizing systemic or ocular side effects. The study was performed to evaluate the potential use of the laser flare-cell meter in monitoring and adjustment of therapy in juvenile uveitis., Patients and Methods: Retrospectively, we monitored 20 children (11 girls and 9 boys) with an age range from 3 to 15 years presenting with juvenile iridocyclitis (10/20), intermediate (5/20) or posterior (5/20) uveitis. During the follow-up period (median 25.2 months, range: 2 to 83 months) multiple clinical controls were performed. We recorded clinical data, present therapy, and measurements using the laser flare-cell meter (Kowa FC-1000)., Results: Laser flare-cell meter measurements were easily obtained and highly reliable even in these young patients. During follow-up, 36 recurrences of intraocular inflammation were detected in 19 eyes. Relapses of inflammation as well as a response to treatment were seen at an early stage. Semiquantitative observations of cells and aqueous flare in the anterior chamber, or visual acuity of patients were less reliable in predicting recurrences., Conclusions: Laser tyndallometry offers a reliable, examiner-independent method to assess intraocular inflammation in children with juvenile uveitis and to adjust the necessary treatment.

Purpose: To evaluate the efficacy of interferon alfa-2a (IFN alfa) in severe uveitis associated with Behçet's disease, that is refractory to steroids and conventional immunosuppressive agents., Patients and Methods: Patients with Behçet's disease (according to the International Study Group criteria), who relapsed despite steroids and immunosuppressive agents, were included in this retrospective study. Ophthalmological examination, laser flare photometry, and fluorescein angiography associated with laboratory tests were performed at regular intervals. IFN alfa (3 millions units thrice a week) was injected subcutaneously., Results: Eight patients (sex ratio: 1) were included between May 1995 and January 1999. The mean age was 29.1 years (14-54 years) and the disease was present between 11 and 167 months before the administration of IFN alfa. IFN alfa was efficient in all cases with a mean follow-up of 22 months (10-55 months). Steroids were tapered from a mean dosage of 47 mg/d to a mean dosage of 8.5 mg/d. Ocular inflammation was controlled and visual acuity improved in all cases. Treatment was generally well tolerated despite a constant but transient flu-like syndrome. IFN alfa was tapered in three patients and stopped in one case without any relapse after five months., Conclusions: Within the limitations of this retrospective study, low-dose IFN alfa seems to be well tolerated, promising in the management of refractory forms of uveitis due to Behçet's disease, and effective in allowing a reduction of steroid dosage. A prospective controlled study is necessary to confirm these preliminary results.