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PubMed1987

Retinal vasculitis: correlation of animal and human disease.

A form of experimental retinal vasculitis was induced in black hooded Lister rats by the inoculation of retinal S-antigen. Comparison of this disease with retinal vasculitis in man showed striking clinical, angiographic and pathological similarities. Clinically disc oedema, periphlebitis and retinal infiltrates were observed with corresponding leakage of dye on fluorescein angiography. Pathologically the disease showed perivascular lymphocytic infiltrates with focal photoreceptor necrosis. These characteristic features make this an ideal model for the study of the pathogenesis of retinal vasculitis in man.

Animals↗

PubMed1988

Retinal vasculitis.

Evidence is now accumulating on both clinical and experimental grounds that the retina is an a priori source of inflammatory activity. Reactive inflammation in the retina may produce many of the clinical signs previously ascribed to uveal inflammation. Autoimmune mechanisms are probably responsible for the majority of cases of retinal vasculitis. Autoimmune retinal vasculitis occurs without other classical signs of inflammatory response in any other parts of the body. When associated systemic manifestations occur they may reflect different underlying immunopathogenic abnormalities. Thus in diseases with predominantly arterial involvement (e.g. systemic lupus erythematosus, polyarteritis nodosa) the retinal arteries bear the brunt of this disease. In Behçet's disease the systemic involvement is usually venous and ocular involvement produces diffuse capillary and venous inflammation with areas of retinal necrosis and major vascular occlusion. The retinal appearances differ from sarcoidosis in which a granulomatous response produces characteristic periphlebitis. Finally, autoimmune retinal vasculitis produces diffuse capillary and venous damage, without any systemic signs. In the next decade the search will be for the identification of the specific antigens initiating these disparate retinal features. Retinal S antigen is a potent antigen, but rhodopsin, interphotoreceptor binding protein, and transducin all need further experimental investigation. Precise documentation will herald the dawn of new therapeutic measures based on a sound immunological fabric.

Adult↗

PubMed2003

Involvement of oxidative and nitrosative stress in promoting retinal vasculitis in patients with Eales' disease.

OBJECTIVES: Eales' disease (ED) is an idiopathic retinal vasculitis condition, which affects retina of young adult males. The histopathological hallmark in ED is the adhesion of leukocytes to the endothelium and the infiltration of these cells into the retinal parenchyma. Phagocyte generated free radicals have been implicated in mediating tissue damage associated with various inflammatory vasculopathies. In the present study, we have investigated the possible role of reactive oxygen species (ROS) and reactive nitrogen species (RNS) in causing retinal tissue damage in ED. DESIGN AND METHODS: 35 patients with ED and 20 healthy control subjects were included in the study. Monocytes (MC) were separated from peripheral blood of the respective study participants. Inducible nitric oxide synthase (iNOS) protein expression was assessed using Western blot and 3 nitrotyrosine (3NTYR) by reversed phase high performance liquid chromatography (RP HPLC). Thiobarbituric acid reactive substances (TBARS) were determined by measuring malondialdehyde (MDA) formed. Superoxide dismutase (SOD) activity was assayed based on the ability of SOD to inhibit auto-oxidation of epinephrine. Iron, copper and zinc content were determined using Atomic Absorption Spectrophotometer. Immunolocalization of iNOS and 3NTYR was performed on the surgically excised epiretinal membranes (ERM) from patients with ED. RESULTS: There was a significant increase in the expression of iNOS, as well as 3NTYR accumulation, diminished SOD activity, elevated lipid peroxides, iron, copper and decreased zinc content in the MC of patients with ED when compared with healthy control subjects. The elevated levels of ROS and RNS products correlated with diminished antioxidant status in patients with ED. Strong immunoreactivity for iNOS and 3NTYR was observed in inflammatory cells and endothelial cells in ERM obtained from patients with ED. CONCLUSIONS: Our findings from this study clearly reveal the involvement of RNS and ROS in the development of retinal vasculitis in ED. Based on our present study and earlier studies we confirm the role of free radicals in mediating retinal tissue damage in ED. Hence we believe selective inhibition of iNOS or supplementation with antioxidants vitamin E and C might be beneficial in controlling retinal vasculitis in patients with ED.

Adult↗

PubMed1989

A point prevalence study of 150 patients with idiopathic retinal vasculitis: 2. Clinical relevance of antiretinal autoimmunity and circulating immune complexes.

This study describes the occurrence of antiretinal antibodies and circulating immune complexes in the sera of a large series of patients with idiopathic retinal vasculitis whose ophthalmological and clinical features are presented in Part 1. Antiretinal antibodies were measured by indirect immunofluorescence and passive haemagglutination, and circulating immune complexes were measured by polyethylene glycol precipitation and Clq binding. The occurrence of antiretinal antibodies and that of circulating immune complexes were analysed in relation to each other, to severity of retinal disease, to the type of associated systemic inflammatory disease, and to the presence of individual features of retinal inflammation. In patients with retinal vasculitis together with systemic inflammatory disease circulating immune complexes were usually accompanied by antiretinal antibodies. However, those patients with antiretinal antibodies in the absence of circulating immune complexes tended to have more severe retinal vasculitis, a feature particularly evident in Behçet's disease (p = 0.028). In patients with isolated retinal vasculitis, severity of disease was associated with antiretinal antibody (p = 0.013), as well as with the occurrence of both antiretinal antibody and circulating immune complexes together (p = 0.010). In the series as a whole there was a tendency for individual features of retinal vasculitis to be associated with antiretinal antibodies unaccompanied by circulating immune complexes; especially in macular oedema (p = 0.028). In isolated retinal vasculitis there was also an additive effect of antiretinal antibodies and circulating immune complexes in relation to disease severity; in contrast, in patients with systemic inflammatory disease, the coexistence of antiretinal antibodies and concluded that both antiretinal autoimmunity and circulating immune complexes may act as immunopathogenetic factors in idiopathic retinal vasculitis but that, in certain patients, circulating immune complex formation seems to protect against the more severe forms of autoimmune retinal inflammatory disease.

Antigen-Antibody Complex↗

PubMed1995

Cranial MRI in idiopathic retinal vasculitis.

Ten patients with clinically isolated idiopathic retinal vasculitis who had a positive family history for multiple sclerosis (MS) or positive typing for HLA B7 underwent magnetic resonance imaging (MRI) of brain and optic nerves in order to establish the frequency of clinically silent lesions. Brain MRI was normal in seven and abnormal in three: one had a single small white matter lesion, two had extensive white matter abnormalities resembling those seen in MS. In two patients a lesion was shown in the optic nerve. These findings suggest that a minority of patients with idiopathic retinal vasculitis have disseminated central nervous system lesions characteristic of MS, the frequency of such changes being less than in patients with isolated optic neuritis.

Adolescent↗

PubMed2004

Elevated intracranial pressure associated with idiopathic retinal vasculitis, aneurysms, and neuroretinitis syndrome.

The idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN) syndrome typically occurs in young patients and may produce multiple retinal macroaneurysms, neuroretinitis, and peripheral capillary nonperfusion. Optic disc edema has been described, but elevated intracranial pressure has not been previously documented. We report a case of a 12-year-old girl who presented with bilateral disc swelling and peripapillary hemorrhage. Brain magnetic resonance imaging (MRI) was normal, but lumbar puncture yielded an opening pressure of 360 mm H2O with normal constituents. Fluorescein angiography delineated saccular aneurysms of the retinal arteriolar vasculature, and IRVAN syndrome was diagnosed. MR venography disclosed poor filling of both transverse venous sinuses. Acetazolamide treatment of 14 months did not alter the fundus findings. IRVAN syndrome may present initially with optic nerve swelling and elevated intracranial pressure with subsequent development of the characteristic retinal vascular abnormalities.

Aneurysm↗

PubMed2001

Retinal vasculitis associated with chickenpox.

PURPOSE: To report retinal vasculitis in a young, immunocompetent Asian female adult with chickenpox. METHODS: Interventional case report. A 32-year-old woman had chickenpox 2 weeks before blurred vision in the left eye. The visual acuity was 20/20 for the right eye and 30/50 for the left eye. The left eye presented keratic precipitates, moderate (2+) cells in the anterior chamber and numerous cells (3+) in the vitreous. The disk was normal. Perivenous exudation was noted mainly in the inferior retina. The sheathed retinal vessels showed late staining but no remarkable leakage on fluorescein angiography. The right eye was normal. RESULTS: After treatment with acyclovir for 10 days, the visual acuity in the left eye improved to 20/20, and the vasculitis resolved. CONCLUSION: Retinal vasculitis may present as a complication of primary varicella infection in an immunocompetent adult.

Acyclovir↗

PubMedDate not supplied

Bilateral Parry-Romberg syndrome associated with retinal vasculitis.

PURPOSE: To describe an unusual case of bilateral progressive facial hemiatrophy (Parry-Romberg syndrome (PRS)) associated with retinal vasculitis. METHODS: In a 37-year-old man with bilateral PRS, retinal vasculitis of the right eye was evident on fundus examination and fluorescein angiography. Right temporalis muscle biopsy and needle electromyography of the masseter muscles were performed. The patient underwent immunosuppressive therapy and retinal laser photocoagulation. RESULTS: Biopsy specimens showed large fibrosis with focal lymphohistiocytic infiltration of the muscle fibers. Electromyographic findings are consistent with a primary muscle disease. Visual acuity improved from 20/25 to 20/20 in the right eye with a follow-up of one year. CONCLUSIONS: The evidence of retinal vasculitis and the histologic findings of facial changes observed in this PRS case could support the pathogenetic model of a chronic inflammatory process as a plausible explanation for progressive facial hemiatrophy.

Adult↗

PubMed2006

[Bilateral retinal vasculitis with arterial aneurysms].

The authors reported unusual and rare condition of bilateral retinal vasculitis primarily affecting the central retinal artery at the nerve head and its 4 main branches. The most striking feature was the presence of the diffuse vitreous cells, occlusion of branch retinal artery, segmental periarterial infiltration, arterial sheating, retinal arterial aneurysms, disc swelling, peripheral retinal non perfusion and their complications. During 13 year's observation and treatment one eye went blind 3 years after initial examination. Second eye started the same clinical course two years after beginning of the disease. To avoid similar devastating course of the disease we started systemic steroids and immunosuppressive therapy, followed by photocoagulation of nonperfused peripheral retina and vitreoretinal surgery. We achieved stabilization of the disease with decreased visual functions. Comprehensive systemic work-up was unrevealing, no clear etiology was identified and diagnosis of idiopathic retinal vasculitis was made.

Adult↗

PubMed2002

Human T-cell lymphotropic virus type-1 associated t-cell leukemia/lymphoma masquerading as necrotizing retinal vasculitis.

OBJECTIVE: To report a case of adult T-cell leukemia/lymphoma (ATL) presenting as a bilateral retinal vasculitis and diagnosed by molecular detection of a rearrangement in the T-cell receptor (TCR) and the presence of the human T-cell lymphotropic virus type 1 (HTLV-1) pol gene in the malignant lymphoid cells. DESIGN: Case report. METHODS: Routine histologic and immunohistochemical analyses were performed on the retinal biopsy specimen before referral to the National Eye Institute. Lymphoid cells associated with granulomatous inflammation infiltrating the retina and surrounding retinal blood vessels were microdissected from the paraffin sections of the retinal biopsy specimen. The polymerase chain reaction (PCR) was performed using primers for the TCR gene and HTLV-1 pol and gag genes. RESULTS: Microscopic examination showed a necrotizing granulomatous retinal vasculitis with a predominant T-cell infiltrate detected by immunohistochemistry. Molecular analysis demonstrated a clonal rearrangement of the TCR and the presence of the HTLV-1 pol gene in the microdissected lymphoid cells diagnostic of ATL. CONCLUSIONS: Necrotizing retinitis and retinal vasculitis are rare manifestations of ATL. Human T-cell lymphotropic virus type 1 infection should be considered in the differential diagnosis of patients from endemic areas who have retinal vasculitis at presentation. This case further demonstrates the usefulness of microdissection and PCR for the diagnosis of ocular disease, including HTLV-1 infection.

Adult↗

PubMed1996

Visual outcome of patients with idiopathic ischaemic and non-ischaemic retinal vasculitis.

This study investigated whether patients with ischaemic retinal vasculitis have a worse visual outcome than those with non-ischaemic disease. A retrospective study was made of 53 patients with idiopathic retinal vasculitis (RV), with minimum 5 year follow-up. Patients were categorised into ischaemic and non-ischaemic groups by fluorescein angiography. Visual outcome was determined by visual acuity at their last attendance. Twenty patients (38 eyes) had ischaemic RV; 33 patients (63 eyes) had non-ischaemic RV. At presentation there was no significant difference between the groups in the proportion of eyes with poor vision (6/60 or less). Ischaemic RV patients had a worse visual outcome than those with non-ischaemic RV: 13 of 38 (34%) eyes in the ischaemic group had a final poor vision compared with 4 of 63 (6%) eyes in the non-ischaemic group (Fisher's exact test, p = 0.0005).

Adolescent↗

PubMedDate not supplied

Tuberculous retinal vasculitis.

A case of central retinal vasculitis associated with culture proven active pulmonary tuberculosis is presented. The ocular condition responded dramatically to systemic isoniazid and ethambutol. The clinical picture of central retinal vein occlusion should alert the physician to the possibility of an inflammatory etiology. Appropriate studies to rule out treatable inflammatory conditions should be ordered.

Female↗

PubMedDate not supplied

[Characteristics of the distribution of major histocompatibility system antigens in retinal vasculitis in patients with Behçet's disease].

The etiology of Behcet's disease (BD) has not been accurately specified until now, however, it is a fact of general knowledge that the genetic factors and environment have an effect on the pathogenesis of the disease. 21 histological antigens (HLA) were typed for 30 Caucasians with BD (14 of them had retinal vasculities, and 16 of them were without it). HLA A and HLA B were determined by using the standard lymphocyte microcytotoxicity technique. The disease was diagnosed in accordance with the criteria of the International Study Group for BD. 1000 healthy donors of the Middle European population were in the control group. The frequency of occurrence of HLA B-5 was reliably higher in patients with retinal vasculitis (85.7%, PR = 5.53) and without retinal vasculitis (68.8%, PR = 4.44) as compared to the controls (15.5%, p < 0.0000). The occlusive retinal vasculitis was found only in BD patients with HLA B-5 (p = 0.0242). The study shows that HLA B-5 is associated with BD and can be a marker of severe retinal vasculitis.

Adult↗

PubMedDate not supplied

[Clinical picture of isolated retinal vasculitis].

Clinical picture of isolated retinal vasculitis (IRV) in 43 patients (25 men and 18 women) aged 22-42 years is analyzed. IRV was bilateral in 88.4% cases and involved the veins. Two forms of disease were distinguished: peripheral (in 75%) and central-peripheral (25%). Both forms were characterized by a high hemorrhagic activity, occlusion of vessels, ischemic zones and neovascularization of the retina and optic disk, but these signs were more frequent in central peripheral form of IRV. IRV is a grave disease leading to poor vision or blindness in 18% cases. The onset is poorly manifest with floating spots and blurred vision; the main symptom is vitreous hemorrhage. Visual acuity was significantly decreased in patients with occlusive IRV in comparison with those without occlusions (0.32 +/- 0.37 vs. 0.77 +/- 0.28). The earliest changes can be detected mainly in the peripheral parts of the retina. This necessitates special attention of ophthalmologist to seemingly negligible complaints of patients and thorough examination of the fundus oculi, particularly of its peripheral sections, which can be performed only on condition of adequate mydriasis. Only timely diagnosis and early pathogenetic therapy can decrease the probability of such complications as hemophthalmos, retinal neovascularization, detachment of retina, and neovascular glaucoma, and prevent disability.

Adult↗

PubMed2002

Optic neuritis and retinal vasculitis as primary manifestations of systemic lupus erythematosus.

Systemic lupus erythematosus (SLE) is a common multisystem disorder. However, retinal vasculitis as a primary manifestation of SLE is uncommon, accounting for only 4% of causes of retinal vasculitis. The postulated mechanism appeared to be vaso-occlusion of the retinal arterioles by thrombosis, with resultant ischaemia. Optic neuropathy in SLE is also rare, with a prevalence of 1%. This is a case report of a young lady who presented to us with retinal vasculitis as her initial presentation of SLE. Interestingly, the pathologic mechanism appeared to be inflammatory and not vaso-occlusive.

Adult↗

PubMed2005

ANCA associated pauci-immune retinal vasculitis.

BACKGROUND: Antinuclear cytoplasmic antibodies (ANCA) are useful diagnostic serological markers for the most common forms of necrotising vasculitis. ANCA associated vasculitides represent distinctive clinicopathological categories--for example, Wegener's granulomatosis, Churg-Strauss syndrome, microscopic polyangiitis, and idiopathic necrotising crescentic glomerulonephritis, collectively known as the small vessel pauci-immune vasculitides. METHOD: Three cases of ANCA associated pauci-immune retinal vasculitis are described. Their systemic features are described and the clinical significance of ANCA as a diagnostic test in relation to retinal vasculitis discussed. RESULTS: These three cases represent a spectrum of clinical features associated with retinal vasculitis. Two cases have evolved into clinical recognisable entities as microscopic polyangiitis. Adherence to the international consensus statement on testing and reporting of ANCA is recommended and the authors speculate that the incidence of microscopic polyangiitis may be underestimated because of the under-recognition of systemic involvement in patients with retinal vasculitis. CONCLUSION: The receipt of a positive ANCA result should always raise the suspicion of a pauci-immune systemic vasculitis and prompt appropriate investigation. The authors emphasise the importance of the evaluation of systemic features in these patients with retinal vasculitis, enabling earlier recognition and thereby preventing significant morbidity and mortality.

Adult↗

PubMed1992

Antibody affinity to retinal S-antigen in patients with retinal vasculitis.

Using a modified enzyme-linked immunosorbent antibody method that included dissociation of antigen antibody complexes with sodium thiocyanate, we examined the functional affinity of antibody to retinal S-antigen in 48 patients with retinal vasculitis and 46 age-matched healthy control subjects. Antibody affinity was markedly lower in patients with retinal vasculitis than in healthy subjects. Low-affinity antibody was more prevalent in acute retinal vasculitis and in patients with normal levels of circulating immune complexes. We found distinct differences between the antiretinal antibodies found in patients with retinal vasculitis and those in control subjects. The association of low-affinity antibody with normal levels of circulating immune complexes may suggest defective regulation of antiretinal autoimmunity and have important pathogenic implications.

Antibody Affinity↗

PubMedDate not supplied

Retinal vasculitis in rheumatoid arthritis: an angiographic study.

The authors studied sixty patients affected by classical or definite rheumatoid arthritis (RA), to evaluate the possible existence of retinal vasculitis, by employing fluorescein angiography. Retinal vasculitis was found in 18% of the examined patients, even if no clinical and ophthalmoscopic signs of retinal vessel inflammation were present. The authors suggest that the retina should be examined in RA patients, since retinal vasculitis may be asymptomatic at the beginning, but can represent one of the possible extra-articular manifestations of the disease.

Adult↗