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Clinical diagnosis of ocular sarcoidosis.

PURPOSE: To assess the value of raised serum angiotensin converting enzyme (ACE) levels in making a clinical diagnosis of ocular sarcoidosis in patients with intraocular inflammation, compatible with sarcoidosis, in whom tissue biopsy is either not practical or not possible. METHODS: The ocular manifestations and clinical course of 22 patients with intraocular inflammation compatible with sarcoidosis and elevated ACE level (including 11 patients with abnormal chest radiograph) were compared with those of a group of 18 patients with intraocular inflammation due to biopsy-proven sarcoidosis. The mean follow-up (+/- SD) was 4.5 +/- 3.4 years in the presumed ocular sarcoidosis group and 7.8 +/- 5.3 years in the biopsy-proven sarcoidosis group. RESULTS: There was no difference in sex, race and age distribution between the two groups. No statistically significant difference could be found between the ocular manifestations seen in each group. The most common finding was retinal vasculitis with panuveitis, seen in 86.4% of the presumed ocular sarcoidosis group and in 83.3% of the biopsy-proven sarcoidosis group. CONCLUSIONS: These results suggest that intraocular inflammation compatible with sarcoidosis in conjunction with raised ACE levels would be accordant with a diagnosis of sarcoidosis in patients in whom histological diagnosis is either not practical or not possible.

Adolescent↗

Tuberculosis: an under-diagnosed aetiological agent in uveitis with an effective treatment.

PURPOSE: To highlight the diversity of clinical presentations with tubercular uveitis in a nonendemic setting, and discuss the diagnostic approach and an effective treatment. METHOD: Descriptive case series. RESULTS: A total of 12 cases of varied presentations of tubercular uveitis diagnosed over a period of 1 year of which six cases are described in detail. Presentations included choroidal tuberculomas, multifocal choroiditis, recurrent granulomatous uveitis, panuveitis with cystoid macular oedema, and serpiginous choroiditis. All cases had a chronic or recurrent course and responded very well to antitubercular treatment. Diagnosis was mainly assisted by positive tuberculin testing. CONCLUSION: A high index of suspicion helps diagnose ocular tuberculosis in areas of low prevalence of the disease. It forms part of the differential diagnosis of any chronic or recurrent uveitis, especially in an at-risk patient. Antitubercular treatment seems highly effective.

Adolescent↗

Risk factors for cystoid macular oedema in patients with uveitis.

PURPOSE: To determine ophthalmologic and systemic factors associated with the presence of cystoid macular oedema (CMO) in patients with uveitis. METHODS: Retrospective cross-sectional study in which 97 consecutive patients with uveitis filled in an extensive questionnaire for the presence of cardiovascular diseases and its risk factors. An analysis of the ophthalmologic and questionnaire data was conducted. RESULTS: CMO was present in 44% (43/97) of patients. Its presence was strongly associated with increasing age (P=0.001) and age at onset of uveitis (P<0.001). For patients older than 50 years, the risk of having CMO was 3.8-fold (95% confidence intervals 1.6-9.0) larger than for younger patients. The most frequent anatomic location of uveitis associated with CMO was panuveitis (49%). Papillary leakage on fluorescein angiography was associated with CMO (P<0.001), independently of other risk factors. After adjustment for age, multivariate logistic regression showed no association between cardiovascular disease and its risk factors and the presence of CMO. CONCLUSIONS: Age, independent of duration of uveitis, was a major risk factor for the presence of CMO in uveitis. A positive correlation between CMO and papillary leakage on angiography was noted.

Adolescent↗

[Oculocerebral malignant non-Hodgkin lymphoma. A report of 5 cases].

Five patients, age 35 to 77, with histologically proven malignent ocular non-Hodgkin's lymphoma (previously known as reticulum cell sarkoma) are presented. The clinical ocular findings (vitreous infiltrates, retinal infiltrates, anterior uveitis) were initially misinterpreted as "panuveitis". The usual antiinflammatory therapie a temporary diminution of the "uveitis" could be observed. Four cases showed a histologically proven CNS involvement. All the patients (except the one in whom the correct diagnosis was made post mortem) received cerebral radiation therapie, including the eyes. Under the radiation therapy the ocular findings disappeared within a few weeks. The longtime prognosis for the eye and quoad vitam is unfortunately not promissing.

Brain Neoplasms↗

[Uveitis in ancylostomiasis].

A 42-year-old patient from Sri Lanka presented with unilateral panuveitis. The only possible cause detected was ancylostoma duodenale, a parasitosis occurring frequently on the Indian subcontinent. The link between this and uveitis, and the therapeutic consequences, are discussed.

Adult↗

Non-infectious causes of uveitis in 70 Swiss children.

Many diseases are linked with uveitis, but few studies have specifically looked at the noninfectious triggers of childhood uveitis in Central Europe. The charts of 70 paediatric patients with non-infectious uveitis admitted to the Department of Pediatrics, University of Bern, Switzerland, between 1983 and 1998 were therefore reviewed. In the patients the age at presentation with uveitis ranged between 0.3 and 16 y, median 8.5 y. Based on the localization, uveitis anterior was diagnosed in most cases (n = 40; 57%), followed by panuveitis (n = 20; 29%) and uveitis posterior (n = 10; 14%). Uveitis was chronic in 54 (77%) and acute in 16 (23%), bilateral in 38 (54%) and unilateral in 32 (46%) cases. An associated condition was noted in 32 (46%) cases: juvenile idiopathic arthritis in 24 cases, sarcoidosis and juvenile spondyloarthropathy in 3 cases, and Sjögren's syndrome and Behçet's disease in 1 case each. In the remaining 38 (54%) patients, no associated condition was diagnosed. It is concluded that in Swiss children, uveitis can be due to a wide spectrum of non-infectious diseases, juvenile idiopathic arthritis being the leading cause. In the majority of the children, no associated condition was recognized.

Acute Disease↗

High incidence of glucose intolerance in Vogt-Koyanagi-Harada disease.

AIMS: To evaluate glucose tolerance of patients with Vogt-Koyanagi-Harada (VKH) disease before systemic corticosteroid therapy, and to assess changes brought on by treatment. METHODS: 20 VKH patients with acute bilateral panuveitis were studied. 20 healthy adults and 11 Behçet's disease patients with active uveoretinitis served as controls. A 75 g oral glucose tolerance test (OGTT) was given in the acute stage of ocular inflammation before systemic corticosteroid therapy. The OGTT was repeated in the convalescent stage of VKH disease in the patients with glucose intolerance before treatment. Insulin response was examined at the same time as the OGTT when possible. RESULTS: 55% of VKH patients (11/20) showed glucose intolerance but no apparent insulin secretion deficiency was detected. Four of seven patients in the convalescent stage showed improvement of glucose tolerance. None of the normal controls or disease controls showed glucose intolerance. CONCLUSION: A high incidence of glucose intolerance was found in the acute stage of VKH disease. However, glucose intolerance improved in most cases after systemic corticosteroid therapy. It is possible that glucose intolerance seen in VKH patients may be related to the autoimmune inflammatory process of this disease.

Adrenal Cortex Hormones↗

Bartonella henselae associated uveitis and HLA-B27.

AIM: To investigate the frequency of HLA-B27 in patients with presumed Bartonella henselae associated uveitis and to describe the clinical characteristics of HLA-B27 positive patients with uveitis and presumed ocular bartonellosis (POB). METHODS: The diagnosis of POB was considered in 19 patients with unexplained uveitis (except for the HLA-B27 association) and high positive IgG (titre >/=1:900) and/or IgM (titre >/=1:250) antibodies against B henselae. In addition to B henselae serology and HLA-B27 typing, all patients underwent an extensive standard diagnostic screening procedure for uveitis and in all cases the results were within the normal limits. The control group consisted of 25 consecutive patients with panuveitis and negative B henselae serology. RESULTS: HLA-B27 was positive in six of the 19 patients (32%) with POB in contrast to the 4% frequency of HLA-B27 in the control group (p=0.03) and 8% prevalence of HLA-B27 in the Dutch population (p=0.003). At the time of positive Bartonella serological testing five of six HLA-B27 positive patients with POB had severe posterior segment involvement with papillitis, macular oedema, and vitreitis. The duration of intraocular inflammatory activity was more than 6 months in five HLA-B27 positive patients. Although four of the six HLA-B27 positive patients had previous recurrent attacks of acute anterior uveitis, the clinical presentation at the time of positive Bartonella serology differed, as illustrated by the involvement of the posterior segment and chronic course of the ocular disease. CONCLUSIONS: The frequency of HLA-B27 in patients with uveitis and serological characteristics of acute infection with B henselae is higher than in the general Dutch population. The findings of this study also suggest a relation between infection with Bartonella species and HLA-B27.

Adolescent↗

HLA-B27 typing in the categorisation of uveitis in a HLA-B27 rich population.

AIMS: To determine whether HLA-B27 typing helps the clinician in the diagnostic examination of uveitis in a HLA-B27 rich population and also whether the clinical picture of HLA-B27 positive unilateral acute or recurrent anterior uveitis (AAU) is distinguishable from the idiopathic negative form. METHODS: During a 3 year period 220 consecutive patients with undetermined uveitis at onset were examined in the Helsinki University Eye Clinic. HLA-B27 antigen was tested for 85% of the patients. Other laboratory or x ray examinations were performed on the basis of the anatomical classification of uveitis and the biomicroscopic features characteristic of uveitis associated systemic diseases. RESULTS: HLA-B27 antigen was found significantly more often in patients with anterior (71%) and acute/recurrent unilateral (79%) uveitis than in patients with intermediate, posterior panuveitis (7%), and chronic (7%) or bilateral (12%) forms. Of the 16 cases of HLA-B27 negative unilateral AAU, five showed biomicroscopic features representing uveitis entities. The remaining 11 cases did not differ in any respect from the cases of HLA-B27 positive unilateral AAU. CONCLUSION: HLA-B27 antigen helps the clinician in the diagnostic examination of unilateral AAU. Positive test results serve as a clue to search for spondyloarthropathies, and negative results indicate the need to look for specific uveitis entities and other systemic diseases. The occurrence of HLA-B27 positivity in conjunction with uveitis entities other than unilateral AAU is of the same level or less than in the population of Finland in general.

Acute Disease↗

A change in ocular involvement in a patient suffering from ankylosing spondylitis and Behçet's disease.

We report a case of a young man suffering from both ankylosing spondylitis and Behçet's disease in whom the character of the ocular involvement changed according to the predominant disease at a given time. When the clinical picture was one of ankylosing spondylitis, only anterior uveitis was observed, while the clinical picture of Behçet's disease occurred with panuveitis and retinal vasculitis. To the best of our knowledge, this is the first report in the English literature of two different patterns of ocular involvement in the same patient with two seronegative arthritides.

Adult↗

Retinitis pigmentosa, Coats disease and uveitis.

PURPOSE: To study the anamnestic immune response to retinal specific antigens of two patients suffering from a rare triad of retinitis pigmentosa, Coats disease and uveitis. PATIENTS: 17-year-old girl presented with an acute episode of panuveitis, and her 19-year-old brother suffered from chronic uveitis. On examination, both patients showed retinal vascular changes and subretinal exudations typical of Coats disease, with bone-spicule pigmentary changes as observed in retinitis pigmentosa. RESULTS: All routine examinations were unrevealing. However, the peripheral lymphocytes from these two siblings gave a specific anamnestic response to retinal antigens in vitro. A stimulation index of 4.6 was obtained when the sister's lymphocytes were stimulated with interphotoreceptor binding protein, IRBP--during the acute stage of the uveitis. The brother's lymphocytes showed a stimulation index of 2.7 towards S-Ag during the chronic phase of his uveitic condition. CONCLUSIONS: These results indicate that autoimmunity towards retinal antigens may play some role in specific types of retinitis pigmentosa. Whether these autoimmune reactions are a primary pathological mechanism or are secondary to the extensive destruction of the photoreceptor layer resulting from the retinitis pigmentosa remains debatable.

Adolescent↗

Isolation of a herpes simplex virus type 2 that is retinovirulent in mice.

The virulence of a herpes simplex virus type 2 (HSV-2) isolated from the urine of a patient (SL) with acquired immunodeficiency syndrome (AIDS) and bilateral acute retinal necrosis (ARN), was investigated in mice. The ratio of plaque forming units (PFU) in fibroblasts to the 50% lethal dose (LD50) of HSV-2(SL) in mice was 10 fold more than the PFU to LD50 ratio of a neurovirulent HSV-2, strain 186. Further, HSV-2(SL) caused retinitis with and without lethal encephalitis in mice inoculated intracranially (i.c.). In contrast, mice inoculated with HSV-2(186) died of encephalitis without ocular disease. HSV-2(SL) was isolated from eye and/or brain tissue 1 to 15 days post i.c. inoculation. Ocular disease progressed from an initial mild chorioretinitis on day 8 to total retinal necrosis with panuveitis by day 11 in mice given 10 PFU of HSV-2(SL) i.c. HSV antigen was detected initially in the cells of the optic nerve and spread into the ganglial cells of the nerve fiber layer, the neurosensory cells of the inner nuclear layer, and the cells of the retinal pigment epithelium (RPE) between days 8 and 10. Thus, this study supports the concept that HSV neurovirulence varies between strains and presents a HSV-2 neurotransmission animal model of ARN.

Acquired Immunodeficiency Syndrome↗

[Uveitis].

Uveitis is a general term that refers to the inflammation of uveal tract, which is an important cause of blindness in young people. It is well known that uveitis can be the initial manifestation of a systemic disease (S.D.), and may appear years before the diagnosis of the primary disease. Uveitis should be integrated in a systemic study with proper testing. Therefore, the diagnosis is a matter for the ophthalmologist and the Specialist in internal medicine. We have made a retrospective study of 71 patients with chronic uveitis or panuveitis. We found 54.9% of primary uveitis and 45.1% of S.D. associated uveitis, most of them with Behçet's disease (16/71) and Ankylosing Spondilytis (7/71). HLA typing of the patients showed a decreased frequency of HLA A1 and HLA A3 antigens and an increased frequency of the HLA B27 antigen, when compared to a Portuguese control population. We confirmed the important role of HLA B27 as an independent susceptibility factor for anterior uveitis. The lowest HLA A3 frequency was observed in the group of S.D. associated uveitis, which could suggest that this antigen may play a role as a factor of resistance to uveitis.

Adolescent↗

[Semiology and classification of uveitis].

Defined as an intraocular inflammation, uveitis may be linked to a systemic disease or represent an isolated entity. Screening for associated extra-ocular manifestations is mandatory in uveitis patients. The localisation of inflammation may be uni- or bilateral, anterior, intermediate or diffuse (panuveitis). Other main elements of the evaluation are the acute or chronic mode and the detection of a granulomatous presentation. In some cases, the examination of intraocular structures may be enough for the diagnosis of uveitis. When a correct approach is taken to the diagnosis of uveitis, causes are identified in approximately 50% of cases and presumed in 25% of patients; in one-fourth of cases, the origin of inflammation remains idiopathic.

Adult↗

[Epidemiology of uveitis].

Uveitis are a large group of inflammatory diseases involving the iris, the ciliary body and the uvea. Multiple causes can be responsible for ocular inflammation that can result either from infectious or autoimmune disease. The incidence varies from 14 to 28/100,000 habitants. According to the anatomical classification, about 30-60% (average 47%) are related to anterior uveitis. 6-30% average 21%) are posterior uveitis, 7-15% (average 12%) are intermediate uveitis and 7-69% (average 20%) are panuveitis. A specific diagnostic can be established in more than 70% in most series. The most frequently diagnosed entities are HLA-B27 related uveitis, acute anterior uveitis in herpes zoster disease, toxoplasmosis, sarcoidosis and pars planitis.

Adolescent↗

[Uveitis in patients with HIV infection].

We carried out a retrospective analysis of 581 patients with uveitis seen over an 11-year period, to determine the prevalence of HIV infection in these patients and to look for associated diseases and the possible causes of uveitis in HIV-infected patients. All patients underwent routine eye examination and most also underwent a general examination and complementary tests. The prevalence of HIV infection was 14.3% (89 patients). Anterior uveitis (62%) was the most frequent, followed by posterior uveitis (22%), panuveitis (12%) and intermediate uveitis (4%). Associated conditions or causes were identified in 88% of the HIV-infected patients, with herpes zoster ophthalmicus the most frequent (43 %), followed by tuberculosis (16%), CMV infection (12%) and toxoplasmosis (10%). Thus, uveitis in HIV-infected patients is frequently associated with opportunistic infections.

AIDS-Related Opportunistic Infections↗

[Clinical variants and immunologic features of rheumatoid uveitis in children of different age].

Variability of clinical manifestations of rheumatoid uveitis, possibility of an atypical course (peripheral or panuveitis), and greater severity and specific features of the disease in case of its early (before the age of 3 years) manifestation are demonstrated on the basis of observations of 79 patients with rheumatoid uveitis. Despite systemic therapy with corticosteroids and cytostatics, no manifest post-steroid immunodeficiency was observed. Moderately expressed gammapathies were detected in 60% cases; hypoimmune states predominated, which were more often detected in children aged under 6 years. No cases of pronounced suppression of functional activity of lymphocytes were observed. Humoral and cellular response to persistent ophthalmotropic infections were observed more rarely than in children with uveitis of other etiology. Positive rheumatoid factor was detected in 9% cases, antinuclear antibodies in 60%. Antibodies to DNA were detected in the sera of 10% examinees, to native and denatured DNA in the lacrimal fluid of 21.2 and 16.7%, respectively. The causes of absence of obvious post-steroid immunodeficiency and relatively low rate of interorgan autosensitization in children with juvenile rheumatoid arthritis and uveitis are discussed. Further investigation of clinical and immunological correlations are needed for improving the prediction of the course of rheumatoid uveitis and development of treatment strategy.

Adolescent↗

Patterns of uveitis in children presenting at a tertiary eye care centre in south India.

PURPOSE: To study the patterns of uveitis in the paediatric age group in a referral eye care centre in south India. MATERIALS AND METHODS: Thirty-one patients 15 years or younger with uveitis, examined in the year 2000, were included in this study. The uveitis was classified according to the anatomical site of ocular involvement and the most probable aetiological factor. The final diagnosis was based on clinical manifestations and results of specific laboratory investigations. RESULTS: A total 31 (6.29%) paediatric uveitis cases were seen among the 493 uveitic cases in the year 2000. The male:female ratio was 17:14. Anterior (9 cases), intermediate (9 cases) and posterior uveitis (9 cases) were seen in equal number. Four patients had panuveitis. Twenty-seven patients had visual acuity of 6/36 or better at presentation. Approximately 25% (8 of 31) patients had cataract secondary to inflammation. Immunosuppressives were administered in 4 patients and one patient required cataract surgery. CONCLUSION: Uveitis in children comprises approximately 6% of uveitis cases in a referral practice in south India. Anterior, intermediate and posterior uveitis are seen in equal numbers. We recommend that intermediate uveitis be ruled out in all cases of anterior uveitis by careful clinical evaluation including examination under anesthesia (EUA) when required.

Adolescent↗