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Intraocular non-Hodgkin's lymphoma treated with systemic and intrathecal chemotherapy and radiotherapy. A case report and review of the literature.

A case of therapy-resistant bilateral intermediate uveitis is presented. Vitreous biopsy and lumbar puncture resulted in the diagnosis of high-grade malignant non-Hodgkin's lymphoma (NHL). Because chemotherapy and radiotherapy continue to yield better results in the treatment of NHL and a cure can be reached in a large group of patients with high-grade malignant NHL, early diagnosis is very important. We therefore recommend early vitreous biopsy in persistent, bilateral, granulomatous intermediate uveitis, especially in older patients. Our patient achieved a complete remission (follow-up period at the time of writing, 18 months after the beginning of polychemotherapy and radiotherapy). The chemotherapy modalities are briefly discussed.

Antineoplastic Combined Chemotherapy Protocols↗

Valsalva retinopathy: diagnostic challenges in a patient with pars-planitis.

PURPOSE: To describe a case of Valsalva retinopathy in a patient with pars-planitis and to discuss its management. METHODS: We present a case of a 19-year-old male with a six-month history of bilateral intermediate uveitis, referred for evaluation of recent-onset spontaneous bilateral vitreous hemorrhages. Clinical evaluation and fluorescein angiography were negative for the presence of neovascularization. On closer questioning the patient revealed that he had his most recent symptoms after heavy weight lifting. RESULTS: The patient was instructed to avoid heavy weight lifting. Over the next two months, vitreal hemorrhages had resolved. However, despite the treatment with systemic prednisone, the patient's intermediate uveitis was still active, and cystoid macular edema had developed. Therefore, we proceeded to systemic immunomodulatory therapy, which controlled the inflammation and preserved vision in each eye. CONCLUSIONS: Although a very rare cause, Valsalva maneuver should be included in the differential diagnosis of bilateral vitreous hemorrhages. Ophthalmologists should advise patients with known eye problems that can predispose to vitreal hemorrhages to refrain from it.

Adult↗

Epidemiological characteristics of uveitis in Switzerland.

Since January 1990, data from uveitis patients have been systematically stored in a computer data bank. During the period from January 1990 to March 1993, 435 new patients (185 female and 250 male, mean age 43 years; range 6-92) were seen at the Uveitis Clinic of the Hôpital Jules Gonin. These 435 patients (630 eyes) were subdivided into anterior uveitis (268 patients--62%), intermediate uveitis (47 patients--11%), posterior uveitis (89 patients--20%) and panuveitis (31 patients--7%). The incidence of uveitis for the referral area considered was calculated to be 17 per 100,000 inhabitants per year. A specific diagnosis was found in 312 cases (72%). The most frequently diagnosed entities were HLA-B27-associated acute anterior uveitis (67 cases--15.4%), uveitis associated with acute herpes zoster ophthalmicus (40 cases--9.2%), toxoplasmosis (39 cases--9%), typical pars planitis (29 cases--6.7%), sarcoidosis (29 cases--6.7%), Fuchs' heterochromic cyclitis (27 cases--6.2%), herpetic anterior uveitis (21 cases--4.8%) and acute retinal necrosis (11 cases--2.5%). Incidence and distribution of most disease entities correspond to those of other European series.

Adolescent↗

[Analysis of aqueous humor in intraocular toxoplasmosis].

BACKGROUND: Despite many clinical examinations and laboratory tests, the cause of uveitis is very often not discovered. The examination of local intraocular antibody production is a valuable additional diagnostic means. PATIENTS AND METHODS: Aqueous humor was obtained from 46 eyes with severe uveitis: retinochorioiditis (n = 27), intermediate uveitis (n = 11), iridocyclitis (n = 5), and panuveitis (3). With the enzyme-linked immunosorbent assay (ELISA) the antibodies against Toxoplasma gondii were measured in aqueous humor and serum and the intraocular antibody production was calculated by the formation of an antibody index (AI). As the AI values are corrected for possible polyspecific immune response, the AI index is more sensitive than the Goldmann-Witmer index. RESULTS: An intraocular antibody synthesis against T. gondii could be confirmed in 20 eyes. The serum antibody concentration against T. gondii was in the reference range in 45 patients and only one patient had a very high increase of antibodies. In nine of 20 eyes, intraocular synthesis of antibodies against T. gondii was unexpected due to the lack of clinical evidence. CONCLUSIONS: The aqueous humor analysis is more reliable than the proof of serum antibodies, and it helps to increase the number of eyes with uveitis in which a specific therapy can be established.

Animals↗

The number of CD8+ T cells and NKT cells increases in the aqueous humor of patients with Behçet's uveitis.

To determine whether there are differences in the immunopathogenesis of different endogenous uveitis syndromes, the phenotypic characteristics of immune cells were analysed among patients with endogenous uveitis. The aetiology of the uveitis included idiopathic recurrent acute anterior uveitis (18 patients), idiopathic intermediate uveitis (13 patients), Behçet's uveitis (17 patients), Vogt-Koyanagi-Harada syndrome (7 patients), and so on. Flow cytometric analysis was performed using immune cells of the aqueous humor and the peripheral blood during the active phase of intraocular inflammation, and monoclonal antibodies to CD3, CD4, CD8, CD14, CD19, CD56, TCR gammadelta, pan TCR alphabeta and Valpha24. CD8+ T cells were predominant in the aqueous humor of the patients with Behçet's uveitis, whereas CD4+ T cells were mainly found in the aqueous humor of patients other than those with Behçet's uveitis. The number of NKT (CD3+CD56+) cells was significantly higher both in the aqueous humor and the peripheral blood of the patients with Behçet's uveitis compared with the other groups (P < 0.05). CD8+CD56+ cells were the predominant subtype of the increased NKT cells in patients with Behçet's uveitis. In addition, intraocular infiltration of CD14+ cells significantly differed among the uveitis patients (P < 0.05). These results suggest that the immunopathogenesis of endogenous uveitis can vary between syndromes, and that CD8+CD56+ NKT cells may play an important role in the immunopathogenesis of Behçet's uveitis.

Adolescent↗

Pattern of uveitis in a referral eye clinic in north India.

PURPOSE: To report the pattern of uveitis in a north Indian tertiary eye center. METHODS: A retrospective study was done to identify the pattern of uveitis in a uveitis clinic population of a major referral center in north India from January 1996 to June 2001. A standard clinical protocol, the "naming and meshing" approach with tailored laboratory investigations, was used for the final diagnosis. RESULTS: 1233 patients were included in the study; 641 (51.98%) were males and 592 (48.01%) females ranging in age from 1.5 to 75 years. The anterior uveitis was seen in 607 patients (49.23%) followed by posterior uveitis (247 patients, 20.23 %), intermediate uveitis (198 patients, 16.06%) and panuveitis (181 patients, 14.68%). A specific diagnosis could be established in 602 patients (48.82%). The infective aetiology was seen in 179 patients, of which tuberculosis was the commonest cause in 125 patients followed by toxoplasmosis (21 patients, 11.7%). Non-infectious aetiology was seen in 423 patients, of which ankylosing spondylitis was the commonest cause in 80 patients followed by sepigionous choroidopathy (62 patients, 14.65%). CONCLUSION: Tuberculosis and toxoplasmosis were the commonest form of infective uveitis, while ankylosing spondylitis and serpiginous choroidopathy were commonly seen as the non-infective causes of uveitis in North India.

Adolescent↗

Pattern of uveitis in a referral uveitis clinic in India.

The pattern of uveitis changes over time with the emergence or identification of new uveitic entities. The aim of this prospective study was to obtain a correct incidence pattern and aetiology of uveitis cases seen in a uveitis clinic. We studied all new uveitic cases seen in a-one year period. A standard clinical protocol was followed for each case. Of the 465 new uveitic cases, anterior uveitis was most commonly encountered (170 cases, 36.5%), followed by posterior uveitis (132 cases, 28.4%), intermediate uveitis (92 cases, 19.8%) and panuveitis (71 cases, 15.3%). The aetiology remained undetermined in majority of the cases (58.7%): anterior uveitis (48.8%), posterior uveitis (45.45%), panuveitis (53.52%). The most common cause was collagen disease (29.4%) in anterior uveitis; toxoplasmosis (30.3%) in posterior uveitis; and Vogt-Koyanagi Harada's disease (25.35%) in panuveitis.

Adolescent↗

Definite multiple sclerosis and uveitis: a two cases report.

Uveitis has infrequently been described as a manifestation of ocular inflammation in patients with multiple sclerosis (MS). Two patients with pre-existing definite MS and uveitis were described. The first patient had features of intermediate uveitis, and the second case had a features of retinal periphlebitis. However, each patient had neurologic signs consistent with MS and a negative laboratory tests for inflammatory and infectious diseases. The presence of neurological involvement with uveitis need a sequential diagnostic search.

Adult↗

Prognosticators for visual outcome in sarcoid uveitis.

PURPOSE: The purpose of the study is to delineate the visual prognosticators in sarcoid-associated uveitis given the current standards of care. METHODS: The records of 60 patients with sarcoid-associated uveitis who were observed for at least 6 months were studied retrospectively. Multivariate regression models using the generalized estimating equations approach to adjust for the correlation between fellow eyes were applied to determine disease, patient, and treatment characteristics that altered the odds of visual rehabilitation. RESULTS: One hundred twelve eyes of 43 women and 17 men who met the inclusion criteria were identified. Seventy-seven percent of patients were white, 15% black, and 8% of Hispanic origin. Uveitis developed in the patients at a mean age of 42 (range, 4-82) years. Of the 112 affected eyes, 81% had granulomatous and 15% nongranulomatous uveitis. Most patients (66%) had anterior or intermediate uveitis alone. Ninety-one percent had chronically smoldering disease; another 7% had recurrent flares, and only 1 patient had a monophasic acute course to her uveitis. Vision-threatening complications developed in many patients, including 58% in whom cystoid macular edema developed and 25% in whom media opacities developed, requiring cataract surgery or vitrectomy or both. Overall, 34% of treated eyes and 51% of patients had final visual acuities that were superior to their acuities at presentation. The factors most significantly associated with a final visual acuity of worse than 20/40 after controlling for potential confounders were as follows: delay in presentation to a subspecialist (odds ratio [OR] = 2.94, P = 0.05), total duration of uveitis (OR = 1.04, P = 0.09), development of cystoid macular edema (OR = 0.37, P = 0.07) or glaucoma (OR = 4.54, P = 0.02), presence of intermediate (OR = 5.00, P = 0.01) or posterior uveitis (OR = 8.33, P = 0.04), and systemic steroid use (OR = 0.30, P = 0.03) were the parameters most strongly correlated with a lack of visual acuity improvement. Delay in presentation to a subspecialist (OR = 20.00, P = 0.01), development of glaucoma (OR = 50.00, P = 0.005), presence of intermediate (OR = 25.00, P = 0.02) or posterior uveitis (OR = 50.00, P = 0.02), black race (OR = 11.11, P = 0.02), (log) visual acuity at presentation (OR = 0.05, P = 0.0001), and use of systemic steroids (OR = 0.07, P = 0.02). CONCLUSION: Multivariate outcomes analysis, particularly after correcting for the correlation between fellow eyes, is a useful analytic tool for optimization of standards of care and for disease risk stratification to aid both physicians and patients.

Adolescent↗

Referral patterns of uveitis in a tertiary eye care center.

OBJECTIVE: To analyze the referral patterns and diagnosis of uveitis during the past decade in a large tertiary eye center. DESIGN: The records of 1237 patients with uveitis referred to the Immunology Service of the Massachusetts Eye and Ear Infirmary from 1982 to 1992 were classified and analyzed. Data regarding sex, race, nationality, referral site, ages at presentation and onset of uveitis, ocular involvement, clinical characteristics, ocular condition, and systemic disease associations were obtained. RESULTS: The mean age at onset of uveitis was 37.2 years; the male-to-female ratio was 1:1.4. Most patients were white (85.8%), born in the United States (83.1%), and referred from within New England (84.7%). Anterior uveitis was most common (51.6%), followed by posterior uveitis (19.4%), panuveitis (16.0%), and intermediate uveitis (13.0%). Chronic (58.3%), nongranulomatous (77.7%), and noninfectious (83.1%) were the most frequent types of uveitis. The most common entities included idiopathic (34.9%), seronegative spondyloarthropathies (10.4%), sarcoidosis (9.6%), juvenile rheumatoid arthritis (5.6%), systemic lupus erythematosus (4.8%), Behçet's disease (2.5%), and the acquired immunodeficiency syndrome (2.4%). CONCLUSION: The appearance of new uveitic entities, such as the acute retinal necrosis syndrome, multifocal choroiditis and panuveitis, birdshot retinochoroidopathy, and acquired immunodeficiency syndrome-related uveitis, and the reemergence of the classic infectious causes of uveitis, tuberculosis and syphilis, have changed the way we approach the diagnosis and management of posterior and panuveitis at the Massachusetts Eye and Ear Infirmary.

Adolescent↗

Uveitis in the southeastern United States.

PURPOSE: Previous epidemiologic studies of uveitis have focused on predominantly Caucasian populations, and none have been based in the Southeast. We analyzed the epidemiology of uveitis among a referral population with a high percentage of African Americans in the United States. METHODS: We evaluated demographic data from 385 consecutive patients referred to the Duke Uveitis Clinic. RESULTS: Of the 385 patients, 120 (31%) were African American and 258 (67%) Caucasian; 237 (62%) were female and 148 (38%) male. The most common diagnoses among the 385 patients were idiopathic panuveitis (64 patients [17%]), idiopathic anterior uveitis (47 patients [12%]), pars planitis (46 patients [12%]), sarcoidosis (44 patients [11%]), and toxoplasmosis (39 patients [10%]). These diagnoses were also the most common among the 120 African American patients: 33 patients (28%) had idiopathic panuveitis, 30 (25%) had sarcoidosis, 10 (8%) had idiopathic anterior uveitis, 8 (7%) had toxoplasmosis, and 6 (5%) had pars planitis. Among the 258 Caucasian patients, the most common diagnoses were pars planitis (39 patients [15%]), idiopathic anterior uveitis (37 patients [14%]), toxoplasmosis (30 patients [12%]), idiopathic panuveitis (28 patients [11%]), and multifocal choroiditis and panuveitis (MCP) (17 patients [7%]). Categorizing diagnoses of all 385 patients by anatomic location, panuveitis was most frequent (148 patients [38%]), followed by anterior uveitis (97 patients [25%]), posterior uveitis (93 patients [24%]), and intermediate uveitis (47 patients [12%]). CONCLUSIONS: The higher frequency of sarcoidosis and idiopathic panuveitis than previously reported is related to our larger African American population base. The racial composition of the Southeast does not, however, account for differences such as our higher percentage of MCP; it is possible that other genetic or environmental factors play a role in this region.

Adolescent↗

Causes of uveitis in a referral hospital in Ankara, Turkey.

PURPOSE: To evaluate the etiology of uveitis and its associated systemic findings in a referral hospital in Ankara, Turkey. MATERIALS AND METHODS: Clinical records of patients in our uveitis department were reviewed retrospectively and 419 eyes of 300 consecutive patients were included in the study. Cases of uveitis due to postoperative endophthalmitis, perforating injuries, and foreign bodies were excluded from the study. The data, including sex, age, clinical presentation, disease course and outcome, ocular involvement, etiology, and associated systemic findings, were entered into a computer database. Pearson chi-square statistical analysis was performed to analyze the data whenever necessary. RESULTS: The study subjects consisted of 162 males and 138 females, with a female to male ratio of 1:1.17 and an average age of 35.7 years. Anterior uveitis was the most common form of uveitis with 43.6%, followed by posterior uveitis (26.6%), panuveitis (20.6%), and intermediate uveitis (9%). Idiopathic uveitis (28.3%) was the most frequent cause of uveitis diagnosed, followed by Behçet's disease (26%) and rheumatic diseases (9%). In 215 (71.7%) out of 300 patients, a specific cause of uveitis with associated systemic findings was identified. Uveitis was attributed to an infectious etiology in only 41 patients (13.6%) of the entire study population. COMMENTS: The etiology of uveitis and its associated systemic findings may vary depending on the geographic distribution of patients and local factors. These results represent those of patients with uveitis referred to hospitals in central Anatolia.

Adolescent↗

Uveitis in HIV-infected patients.

PURPOSE: To determine the prevalence of HIV infection and to find out the possible causes (associated conditions) of uveitis in HIV-infected patients. METHODS: We retrospectively analyzed the data of 581 patients with uveitis diagnosed over an 11-year period. All patients received a routine eye examination and most of them a general examination as well as complementary tests. RESULTS: The prevalence of HIV infection was 14.3% (89 patients). Anterior uveitis (62%) was the most frequent form, followed by posterior uveitis (22%), panuveitis (12%) and intermediate uveitis (4%). Associated conditions or causes were found in 88% of these 89 patients, the most frequent being Herpes zoster ophthalmicus (43%), tuberculosis (16%), CMV infection (12%) and toxoplasmosis (10%). CONCLUSIONS: In HIV-infected patients uveitis is frequently associated with opportunistic infections.

Adult↗

Pediatric uveitis in southern Turkey.

Uveitis in the pediatric population is not so common, and the etiology is different from adults. In the present study the charts of the patients with onset of uveitis at 16 years of age or younger were reviewed in order to analyze the etiology in pediatric patients with uveitis, and to compare the results with other studies carried out on different populations. The charts of 90 cases, followed at the Uvea-Immunology Clinic of the Cukurova University Medical Faculty Department of Ophthalmology, between January 1987 and February 1996 were reviewed retrospectively. There were 47 girls, 43 boys, aged 5-20 (13.64 +/- 4.24) in the study group. The average age at onset of uveitis was 12.20 +/- 4.81, and the follow-up period was 9-98 months (21.80 +/- 11.13). Of the 90 patients 31 (34.4%) had panuveitis, 30 (33.3%) had anterior uveitis, 21 (23.3%) had posterior uveitis and 8 (8.9%) had intermediate uveitis. Only 2 (9.5%) of the patients with posterior uveitis were considered idiopathic after extensive laboratory and clinical work-up, whereas idiopathic cases constituted 48.4% of panuveitis, and 46.7% of anterior uveitis cases. Of the 90 patients an associated condition could be found in only 59 (65.6%) patients. Of these 59 patients 23 had toxoplasmosis (39%), which constituted the most common associated condition in this study. The second most common underlying cause was Behçet's disease (17%), followed by pars planitis (13.6%), Fuchs' heterochromic iridocyclitis (8.5%), JRA (5.1%), leukemia (5.1%), and herpetic eye disease (5.1%). There were single cases with Reiter, toxocariasis, traumatic uveitis, and sympathetic ophthalmia. Environmental, cultural and genetic factors may be accountable for the differences in relative frequencies of some of the associated conditions between our findings and those of previously published studies in patients with pediatric uveitis.

Adolescent↗

Humoral and cellular immune responsiveness to human S-antigen in uveitis.

Purified human retinal S-antigen (S-ag) was used to investigate the occurrence of humoral and cellular autoimmune reactions against S-ag in uveitis patients. With a sensitive ELISA method anti-S-ag antibodies could be detected in the sera of 28% of the uveitis patients. No difference was found between patients with posterior or panuveitis (31 out of 117 positive) and patients with anterior or intermediate uveitis (16 out of 52 positive). Similar frequencies and levels of anti-S-ag autoantibodies were also found among healthy controls (6/20) and patients who had undergone cataract surgery (6/17). Immunoblotting with purified S-ag and with whole human retinal extract confirmed the presence of anti-S-ag antibodies in uveitis and control sera. Moreover, antibodies against various other retinal proteins could also be demonstrated in patients and controls, without being particularly enhanced in uveitis. The cellular immune responsiveness was tested by measuring the production of migration inhibitory factor (MIF) during overnight culture of peripheral mononuclear cells with the antigen. None of 18 healthy controls responded, whereas 17 positive reactions were observed in the group of 44 uveitis patients. The highest frequencies were found in patients with posterior (5/12) or pan- (7/12) uveitis, while of the responders with anterior (2/8) or intermediate (3/12) uveitis, three had disorders affecting the retina. Thus, cellular autoimmune responsiveness to S-ag is apparently associated with posterior and pan-uveitis, and might also occur in non-uveitic retinal disorders, whereas the occurrence of anti-S-ag antibodies is probably not at all pathognomic for uveitis.

Adolescent↗

Uveitis associated with human T-cell lymphotropic virus type I.

Seroepidemiologic, clinical, and virologic studies were performed to determine whether human T-cell lymphotropic virus type I was closely associated with uveitis in two hospitals. One hospital was in an endemic area of the virus (Miyakonojo, Miyazaki) and the other hospital was in a less endemic area (Kurume). In the endemic area, the seroprevalence of the virus in patients with uveitis without defined causes (35.4%, 62 of 175 patients) was significantly higher than that in patients with nonuveitic ocular diseases (16.1%, 42 of 261 patients), or in patients with uveitis with defined causes (10.3%, eight of 78 patients). The seroprevalence in younger patients (20 to 49 years of age) with uveitis without defined causes in the area was 44.8% (30 of 67 patients), whereas it was only 9.3% (ten of 107 patients) in the other two groups. A similar observation was recorded even in the less endemic area (Kurume). Because the seroprevalence of the virus in the general population is known to be low in younger patients and to increase with age, these findings were interpreted to indicate that the association of human T-cell lymphotropic virus type I with uveitis was significant. Most patients, particularly those aged 20 through 49 years, had an intermediate uveitis characterized by a moderate inflammation in the vitreous body accompanied by an iritis and retinal vasculitis. The ocular symptoms in the patients differed from those of other types of uveitis common in Japan (Behçet's disease, Vogt-Koyanagi-Harada's disease, and toxoplasmosis, for example).(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

Chronic granulomatous anterior uveitis associated with multiple sclerosis.

A retrospective review is presented of the medical records of seven female patients with uveitis associated with definite multiple sclerosis. Six of the patients had severe bilateral chronic granulomatous anterior uveitis, which in four cases resulted in extensive posterior synechiae and scarring of the peripupillary iris. One patient had bilateral intermediate uveitis with unilateral posterior synechiae. Severe chronic granulomatous anterior uveitis associated with multiple sclerosis has a predilection for women, can precede neurological symptoms and can be controlled with topical steroids.

Administration, Topical↗

Posterior sub-Tenon's steroid injections for the treatment of posterior ocular inflammation: indications, efficacy and side effects.

PURPOSE: Posterior sub-Tenon's steroid injections (PSTSI) are a standard drug delivery method used for the treatment of chronic uveitis of the posterior segment. The aim of this study was to analyse the indications, efficacy and complications of PSTSI in the treatment of chronic uveitis. METHODS: During the period 1990-1994, 53 (9.5%) of 558 patients (58 eyes) followed up in the uveitis clinic received a total of 162 PSTSI of triamcinolone acetonide 40 mg in the superior quadrants. Indications for treatment were vision inferior or equal to 0.7 and/or intolerable visual disturbance. Only patients in whom PSTSI were the only treatment parameter changed were analysed. Among the main parameters analysed were visual acuity, aqueous laser flare photometry, intraocular pressure (IOP) and complications. RESULTS: Anatomical location of uveitis was as follows: anterior HLA-B27-related uveitis with CME (1 patient/1 eye), intermediate uveitis (28/32), posterior uveitis (10/10) and panuveitis (14/15). Mean duration of follow-up was 448+/-57 days. Visual acuity improved significantly from 0.40+/-0.03 to 0.79+/-0.07, with 59.4% of eyes having a gain of 2-5 Snellen lines and 18.7% a gain of >5 lines. Mean aqueous flare photometry decreased significantly from 29.6+/-3.5 to 13.6+/-2.2 photons/ms. Mean IOP increased significantly from 13.6+/-0.5 to 18.5+/-0.8 mm Hg with a rise of pressure >8 mm Hg in 23 cases (36%), transient in 16 cases, but chronic in 6 cases, needing filtering surgery. Partial superior ptosis was seen in two cases and cataract progressed in seven cases. CONCLUSION: PSTSI are very effective in restoring visual acuity in chronic uveitis of the posterior segment, without systemic complications, but at the expense of intraocular hypertension, a complication that was found more frequently than expected.

Adolescent↗