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PubMed1977

Circulating immune complexes in retinal vasculitis.

Seventeen patients with retinal vasculitis, eleven with the peripheral type (Eales' disease) and six with the central type, were investigated to detect the presence of circulating immune complexes (IC) which might then be related to the pathogenesis of their disease. A systemic disease process was identified in six. IC in serum were inferred by the presence of complement (C) activation, rheumatoid factor, Clq or monoclonal rheumatoid factor precipitins, anticomplementary activity, elevated cryoglobulins, inhibition of erythrocyte-antibody (IgG-EA) rosette formation, increased numbers of peripheral blood lymphocytes bearing surface Ig, and spontaneous neutrophil chemotatic activity in plasma. Two or more parameters were positive in thirteen of seventeen patients, with chemotactic activity (69%) and inhibition of EA-rosette formation (59%) being the most frequently positive tests. No immunological differences were detected between the peripheral and central retinal-vasculitis groups. Several IC systems may operate in a give patient.

Adult↗
PubMed1991

Retinal vasculitis in Lyme borreliosis.

Three patients with retinal vasculitis are reported who were found to be seroreactive for Lyme borreliosis. Careful investigation revealed no other apparent etiology for the angiitis, and improvement of the retinal vasculitis on tetracycline therapy was documented by fluorescein angiography in one of them. Two cases of retinal vasculitis were presented at the International Conference on Lyme Borreliosis in Stockholm 18-21 June 1990, and two cases of cerebral vasculitis due to Borrelia burgdorferi have been published. To our knowledge, this is the first published report of retinal vasculitis occurring in patients seroreactive for Lyme borreliosis. Although further investigation will be necessary to prove a cause-and-effect relationship, ophthalmologists encountering patients with otherwise unexplained cases of retinal vasculitis, or Eales disease, are encouraged to study these patients carefully for the possibility of Borrelia burgdorferi infection.

Adult↗
PubMed1999

The pituitary-adrenal axis in idiopathic retinal vasculitis.

AIMS: To determine whether patients with idiopathic retinal vasculitis have altered production of cortisol and dehydroepiandrosterone sulphate (DHEA-S), and whether differences in these variables occur between those who are sensitive (SS) and resistant (SR) to steroids. METHODS: 20 patients with retinal vasculitis (off treatment) and 10 control subjects were prospectively recruited. Morning cortisol and DHEA-S levels were measured, and cortisol secretion rates and short synacthen tests (SST) carried out in patients before treatment, when on prednisolone 20 mg/day, and in controls. RESULTS: There were no differences in any variables between patients and controls. For retinal vasculitis patients pretreatment, the SST was lower in SR patients (p=0.02). More of the SR patients had ischaemic retinal vasculitis ( p<0.001). CONCLUSIONS: Cortisol and DHEA-S are not involved in the pathogenesis of retinal vasculitis. SR in retinal vasculitis may be associated with a defective hypothalamic-pituitary-adrenal axis.

Adult↗
PubMed1996

Primary retinal vasculitis. Systemic associations and diagnostic evaluation.

BACKGROUND: Inflammation of the retinal vasculature without an infectious etiology, systemic disease association, or concomitant ocular disease is termed primary retinal vasculitis, and can result in severe and permanent vision loss. Patients with primary retinal vasculitis usually are subjected to an extensive but unrewarding diagnostic evaluation. This study was undertaken to determine the value of such a diagnostic workup, and to determine whether systemic diseases develop in these patients during the course of their illness. METHODS: The clinical records of 25 patients seen between 1984 and 1994 with the referring diagnosis of primary retinal vasculitis were reviewed retrospectively. Recorded data included patient age, sex, race, medical history, medications, visual acuity, extent of retinal disease, and the results of their diagnostic evaluations. RESULTS: On presentation, none of the patients had an underlying systemic disease attributable as the cause of their retinal vasculitis. Review of systems was suggestive of an underlying systemic disease in 1 of 25 patients. Diagnostic evaluation in this patient showed a positive antinuclear antibody value and a double-stranded DNA, suggestive of systemic lupus erythematosus. Subsequently, systemic lupus erythematosus was diagnosed based on the development of other diagnostic criteria. The review of systems was not suggestive of an underlying systemic disease in 24 of 25 patients. False-positive diagnostic test results were obtained in 5 (20.8%) of these 24 patients. No underlying systemic disease associated with the patients' retinal vasculitis subsequently developed in any of these five patients (mean follow-up, 4 years). CONCLUSION: Few diagnostic tests should be ordered in patients with retinal vasculitis in the absence of a medical history suggestive of underlying systemic disease.

Adolescent↗
PubMed1985

[Retinal vasculitis in lupic disease].

Three cases of retinal vasculitis in SLE-type diseases are reported. The first was central retinal vein occlusion occurring during clinical remission of SLE in a 55 year old black female. Prednisone maintenance therapy was unchanged and visual loss rapidly regressed with heparin therapy. The second case was a 33 year old black female in whom SLE was discovered following relapsing bilateral optic neuritis. A progressive visual improvement was obtained with high dose of prednisone (1 mg/kg/day). The third cas was a 17 year old white girl with retinal vasculitis. She had an unclassified connective tissue disease inaugurated by optic neuritis at the age of 10. High dose prednisone (1 mg/kg/day) was effective on the visual loss. Retinal vasculitis lesions in SLE and their therapy are reviewed.

Adult↗
PubMed2002

Rapid seroconversion to Treponema pallidum and HIV positivity in a patient with retinal vasculitis.

A patient with idiopathic retinal vasculitis was found to have rapid conversion of serological tests for Treponema pallidum and HIV. The diagnosis was confirmed by Venereal Disease Research Laboratory (VDRL) testing and dark ground illumination for T. pallidum, and enzyme-linked immunosorbent assay (ELISA) and Western blot assay tests for HIV. Following treatment with intravenous penicillin, the fundus lesions resolved and visual acuity recovered from inaccurate light projection to 6/24 in the right eye and from counting fingers close to face to 2/60 in the left eye. Serological tests for T. pallidum in patients with concurrent HIV infection may be unpredictable. Hence, it is important to repeat these tests even in the early treatment phase of patients with retinal vasculitis who have shown initial seronegativity. This enables earlier diagnosis and initiation of specific treatment.

Adult↗
PubMed2001

PCR-positive tubercular retinal vasculitis: clinical characteristics and management.

BACKGROUND: Inflammation of retinal vessels is a known association of systemic tuberculosis. Patients with retinal vasculitis are subjected to extensive but unrewarding systemic workup. Polymerase chain reaction (PCR) is now commonly used to detect DNA of infective organisms including Mycobacterium tuberculosis. This study was undertaken to characterize the clinical characteristics of PCR-positive tubercular retinal vasculitis, so as to determine the clinical presentation, associated systemic features, management, and course of this form of vasculitis. METHODS: The clinical records of 13 patients seen between 1997 and 1999 with the diagnosis of PCR-positive tubercular retinal vasculitis from the aqueous or vitreous humor were reviewed. Recorded data included age, sex, race, visual acuity, anterior and posterior segment findings, and results of diagnostic evaluations. All received antituberculosis therapy with or without concomitant corticosteroids. Laser scatter photocoagulation was done in eyes with neovascularization. One eye with vitreous hemorrhage was subjected to pars plana vitrectomy. RESULTS: There were 9 (69.2%) male and 4 (30.7%) female patients with a median age of 20 years. The disease was bilateral in seven. The most consistent finding was the presence of vitritis in all the eyes followed by vitreous snowball opacities in 17 eyes (89.4%), neovascularization in 11 eyes (57.8%), retinal hemorrhages in 10 eyes (52.6%), neuroretinitis in 10 eyes (52.6%), focal choroiditis in 9 eyes (47.3%), vitreous/preretinal hemorrhage in 5 eyes (26.3%), and serous retinal detachment in 3 eyes (15.7%). Over a median follow-up of 12 months, all showed resolution of vasculitis with no recurrences. CONCLUSIONS: Polymerase chain reaction-positive tubercular retinal vasculitis had varied associated fundus findings. Its recognition is important so as to order only relevant diagnostic tests.

Adolescent↗
PubMed1997

Hyperimmunoglobulinemia D in idiopathic retinal vasculitis.

BACKGROUND: We examined four patients who exhibited both idiopathic retinal vasculitis and elevated serum IgD levels. Uveitis caused by Behçet's disease is also associated with high levels of serum IgD. Therefore, the clinical features of these patients were investigated and the possible relationship between retinal vasculitis and elevated serum IgD was examined after undertaking a study of increased IgD levels in patients diagnosed with uveitis. METHODS: The study population was composed of 110 patients: 49 with Behçet's disease, 15 with sarcoidosis, 10 with Vogt-Koyanagi-Harada disease, and 36 with other forms of uveitis. IgD measurements were performed using modifications of the latex photometric immunoassay. Surface IgD (sIgD) expression in peripheral lymphocytes was determined by immunofluorescence, and the correlation between serum IgD levels and the percentage of sIgD-positive cells was examined. RESULTS: Twelve of the 110 patients had an elevated serum IgD. Eight of the 12 had Behçet's disease, and 4 were diagnosed with idiopathic retinal vasculitis. These 4 patients were HLA-A24+ females whose ages ranged from 8 to 25 years. A linear correlation between the serum IgD levels and the percentage of sIgD-positive cells was found. CONCLUSION: Hyperimmunoglobulinemia D state was found in Behçet's disease and idiopathic retinal vasculitis. These diseases may represent a new clinical entity characterized by signs of retinal vasculitis and hyperimmunoglobulinemia D that results from abnormal B cell activation and immune complex-mediated responses.

Adolescent↗
PubMed1982

Anti-retinal autoimmunity and circulating immune complexes in patients with retinal vasculitis.

Sera from 44 patients with isolated retinal vasculitis (RV), 38 patients with retinal vasculitis accompanying systemic inflammatory diseases (RV + SID), and 33 patients with a similar range of systemic inflammatory diseases without eye involvement (SID alone) were assayed for circulating immune complexes (CIC) and for anti-retinal autoantibodies. CIC were present in 41% of patients with isolated RV and 55% of patients with RV + SID, whilst anti-retinal antibodies were present in about 70% of all patients with RV. 42% of those with SID alone had CIC and 30% of those with SID alone had retinal autoantibodies. Titres of anti-retinal antibodies were higher in patients with RV than in those with SID alone. In isolated RV there was an inverse relation between pronounced retinal autoimmunity and the occurrence of CIC--i.e., the more severe autoimmune retinal disease occurred in CIC-negative patients. Most patients with RV + SID tended to have mild or moderate retinal disease accompanied by both retinal autoantibodies and CIC, but severe retinal disease occurred in CIC-positive patients who did not have circulating anti-retinal antibodies. Patients with SID alone had high titres of retinal antibodies only when they were CIC-positive. It is suggested that the formation of CIC, possibly of an idiotype/anti-idiotype nature, may be a compensatory mechanism accompanying anti-retinal autoimmunity and that an imbalance between autoimmunity and immune complex formation may be an important predisposing factor in the development of retinal inflammatory disease.

Antibody Specificity↗
PubMed2000

Retinal vasculitis occurring with common variable immunodeficiency syndrome.

PURPOSE: To report severe retinal vasculitis causing decreased vision in three patients with the common variable immunodeficiency syndrome. METHOD: Case report. Three patients with common variable immunodeficiency syndrome developed decreased vision secondary to retinal vasculitis. Fluorescein angiography was performed in all three patients. Peribulbar injections were given in one patient, and two patients were treated with oral steroids and cyclosporin. RESULTS: All three patients were young and had classic common variable immunodeficiency syndrome. Bilateral retinal vasculitis and diffuse retinal edema were present in all three patients, and two patients had retinal neovascularization in the absence of ischemia. No evidence of intraocular infection was present, and none was detected systematically. Visual acuity decreased in five of the six eyes and was responsive to treatment in only one patient (both eyes). CONCLUSION: Retinal vasculitis may be another autoimmune manifestation of common variable immunodeficiency syndrome.

Capillary Permeability↗
PubMedDate not supplied

[Retinal vasculitis in Behçet's disease].

Behçet's disease (BD) is a multisystemic disorder characterized by primary vasculitis of unknown etiology. We studied retinal vasculitis in 35 patients with ocular form of BD. All patients fulfilled the International Study Group criteria for BD. Mean age was 30.8 +/- 6.5 years (19-45 years, mean follow-up period 4.1 +/- 1.29 years (3-10 years. The disease occurred 3.3 times more often in men than in women (p = 0.0003). Retinal vasculitis was diagnosed in 26 (74.3%) of 35 patients with ocular form of BD. Retinal vascular changes characteristic of BD were bilateral in 96.1% cases and involved both arteries and veins. The earliest changes were detectable only by fluorescent angiography. Diffuse capillary leakage was observed in all cases during the active period of disease, involving the posterior pole. The disk and macular capillaries were involved most of all. Late staining of vasculature was observed in 68.6% patients. No ruptures of retinal vessels, retinal neovascularization, or areas of retinal nonperfusion were observed (except one case with occlusion of a branch of the central retinal vein), which are usually seen in other types of retinal vasculitis. Visual acuity was significantly decreased in patients with retinal occlusive vasculitis in comparison with patients without it (0.04 +/- 0.09 vs. 0.3 +/- 0.3, p = 0.029).

Adult↗
PubMed1981

Autoimmunity and circulating immune complexes in retinal vasculitis.

This paper presents the results of a point-prevalence study of circulating autoantibodies and immune complexes in 44 patients with isolated retinal vasculitis (RV alone), 38 patients with retinal vasculitis accompanied by systemic inflammatory disease (RV + SID), and 33 patients with a similar range of systemic inflammatory disease but without eye involvement (SID alone). In isolated retinal vasculitis, antiretinal antibodies, non-retinal antibodies, and circulating immune complexes each showed a prevalence of about 50 per cent, but there was an inverse relationship between marked antiretinal autoimmunity and the occurrence of circulating immune complexes. In SID alone, antiretinal antibodies (ret-AB) were of lower prevalence (about 20 per cent) and occurred only in patients who had circulating immune complexes (CIC). Patients with RV + SID fell into two principal categories: (a) ret-AB with CIC, and (b) ret-AB without CIC. In isolated retinal vasculitis, the occurrence of high levels of retinal autoimmunity in the absence of circulating immune complexes was associated with the more severe retinal disease, while in those RV + SID patients who did not express autoimmunity, the more severe retinal disease was associated with the presence of circulating immune complexes. It is suggested that the formation of circulating autoimmune complexes, possibly of an idiotypic: anti-idiotypic character, may be a 'risk' mechanism for limiting retinal-specific autoimmunity, and that certain patients with severe retinal vasculitis may undercompensate or overcompensate in this way.

Antigen-Antibody Complex↗
PubMed1981

Immunological investigations in retinal vasculitis.

Immunological investigations have been performed in eighty patients with retinal vasculitis. 50 per cent of patients with retinal vasculitis associated with systemic inflammatory disease have raised circulating immune complexes, abnormal complement abnormalities, and antibodies to retinal S antigen. Patients with isolated retinal vasculitis demonstrate similar abnormalities when vascular leakage is the predominant manifestation, but a higher incidence (89 per cent) of retinal antibodies and a lower incidence of immune complexes (22 per cent) are found if reduced retinal perfusion is present. A rise in titre of antibodies to smooth muscle occurred in several patients before the recurrence of uveitis, indicating that this test may predict a clinical relapse.

Adolescent↗
PubMed1989

A point prevalence study of 150 patients with idiopathic retinal vasculitis: 1. Diagnostic value of ophthalmological features.

This paper describes the ophthalmological features of 150 patients with idiopathic retinal vasculitis, 67 of whom had isolated retinal vasculitis (RV) and 83 had RV associated with systemic inflammatory disease (RV + SID). The diagnosis of retinal vasculitis was made by ophthalmoscopy and fluorescein angiography, and patients with any identifiable cause (infection, ischaemia, or malignancy) were excluded from the study. Patients with isolated RV tended to have peripheral vascular sheathing, macular oedema, and diffuse capillary leakage. Those with RV accompanying Behçet's disease often had branch vein retinal occlusions and retinal infiltrates together with macular oedema and diffuse capillary leakage; the retinal infiltrates were pathognomonic for Behçet's disease. In sarcoidosis the retina typically showed features of periphlebitis associated with focal vascular leakage. Patients with uveomeningitis, multiple sclerosis, arthritis, or systemic vasculitis showed diffuse retinal capillary leakage associated with a mixture of the other features. Poor visual function was particularly associated with macular oedema and branch vein retinal occlusion, while the retina appeared to 'withstand' the impact of vascular sheathing, periphlebitis, or neovascularisation alone. Within the limitations of a point prevalence study it was concluded that different patterns of retinal vasculitis occur in different systemic inflammatory diseases, and that in isolated retinal vasculitis there is a particular association between peripheral vascular sheathing, macular oedema, and diffuse capillary leakage. In Part 2 we describe the results of examining the sera of these patients for the presence of antiretinal antibodies and circulating immune complexes.

Adolescent↗
PubMed1988

A longitudinal study of clinical and immunological findings in 52 patients with relapsing retinal vasculitis.

Fifty-two patients with retinal vasculitis--26 with idiopathic disease and 26 with associated systemic inflammatory disease--were followed up for periods ranging from six months to 12 years. The aim of the study was to determine the relationship between relapse of uveitis, visual outcome, and the occurrence of circulating immune complexes (CIC) and antiretinal antibodies. In a total of 69 relapses, CIC were increased in one-third of patients and antiretinal antibodies in one-half. In those 34 patients who expressed antiretinal antibodies 27 (79%) of the relapses were characterised by antiretinal antibodies in the absence of raised CIC levels (p less than 0.01). These findings support our previous hypothesis that CIC may have a protective role in autoimmune retinal vasculitis and that antiretinal autoimmunity is of pathogenetic importance in relapse. In individual patients the visual outcome was not related to the number of relapses or to the CIC-autoantibody pattern, suggesting the operation of additional features which merit identification.

Adolescent↗
PubMedDate not supplied

Retinal vasculitis due to secondary syphilis.

BACKGROUND: Retinal vasculitis is one of the manifestations of ocular syphilis. CASE: A 29-year-old man was referred to our hospital with the complaint of sudden visual loss in the left eye lasting more than three weeks. OBSERVATIONS: Ophthalmoscopic examination showed retinal hemorrhages, edema, and sheathing of large retinal arteries and veins. Fluorescein angiography revealed extensive occlusion of the affected retinal arteries, veins, and capillaries. Little evidence of uveitis or vitritis was observed. The fluorescent treponemal antibody-absorption test was positive, and the Treponema pallidum hemagglutination titer was 1:10,240. The treatment with penicillin was effective, leading to resolution of the retinal hemorrhages and edema, although occlusion of the retinal vessels persisted. CONCLUSIONS: Vascular occlusion occurred simultaneously in large retinal arteries, arterioles, and capillaries as well as in segments of retinal veins, resulting in irreversible changes in the vascular walls.

Adult↗
PubMed1988

Acute multifocal hemorrhagic retinal vasculitis.

The authors present a series of seven patients with acute visual loss associated with mild anterior uveitis, multifocal retinal vasculitis, retinal capillary nonperfusion, retinal hemorrhage, disc swelling, and vitreitis. Oral prednisone was of some benefit in these patients and oral acyclovir was generally ineffective. Neovascular complications including retinal, disc, choroidal, and iris new vessels were common, requiring photocoagulation in five patients. Horseshoe tears developed in two patients in zones of uninvolved retina but retinal detachment did not occur. The etiology remains unknown, although it may represent either a localized ocular form of Behçet's disease or other systemic syndrome, infection with a herpes group virus other than zoster varicella virus, or a manifestation of an undefined infectious agent.

Acute Disease↗