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A 57-year-old Dutch woman, who had lived and worked in Tanzania for 26 years, developed serious, progressive bilateral retinitis after suffering from a fever of unknown origin. Fundus abnormalities in both eyes suggested that the woman had been infected with Rift Valley fever virus. Serologic studies confirmed this suspicion. White macular lesions accompanied to extensive vasculitis appear to be specific for Rift Valley fever retinitis.
A male rhesus macaque was found to have what appeared to be numerous platelet-fibrin emboli in retinal vessels in the perimacular area. Indocyanine green (ICG) dye fluorescence and fluorescein angiograms of the fundus demonstrated leakage of fluorescein, but not ICG, from the involved arterioles. Histopathologic changes in the eyes included occlusion of retinal and choroidal vessels with platelet-fibrin emboli, inner retinal ischemia, ischemic injury to the parafoveal capillary bed distally to occlusion of precapillary arterioles, and retinal exudate limited to the regions of capillary damage. Differential leakage of fluorescein may be explained by the difference in binding affinities of the 2 dyes to blood protein: 20% to 40% of the circulating fluorescein is unbound, and 98% of ICG is bound to serum albumin. Simultaneously or serially performed angiograms with fluorescent probes of different sizes might be used to obtain a qualitative measure of vascular integrity in persons with embolism, diabetic retinopathy, sickle cell retinopathy, vasculitis, and other disorders known to produce focal retinal and choroidal vascular occlusion.
BACKGROUND: The etiology of HIV-related retinal microangiopathy syndrome is yet unknown. Several authors postulate direct endothelial-cell infection, an immunocomplex vasculitis caused by HIV-related hypergammaglobulinemia or an increased serum concentration of endothelin as its origin. PATIENTS: 118 patients infected by HIV-1 have been examined (CDC I: 1; CDC II: 42; CDC III: 7; CDC IV: 68). 49 out of them were also infected by hepatitis-C-virus (CDC I: 0; CDC II: 16; CDC III: 4; CDC IV: 29). RESULTS: 26 out of 49 patients with hepatitis-C-co-infection showed HIV-related retinal microangiopathy syndrome (CDC I: 0/0; CDC II: 8/16; CDC III: 2/4; CDC IV: 16/29). In 69 patients without hepatitis-C-infection, HIV-related retinal microangiopathy syndrome was found five times (CDC I: 0/1; CDC II: 2/26; CDC III: 0/3; CDC IV: 3/39). CONCLUSION: Co-infection with hepatitis-C-virus is supposed to enhance the development of retinal microangiopathy syndrome in patients infected by HIV-1.
PURPOSE: Eales' disease (ED) is an idiopathic retinal inflammatory disease, which affects the retina of young adult males. Oxidative stress has been associated with the development of various inflammatory diseases. Accumulation of DNA oxidation products has been considered as an early biomarker for assessing oxidative tissue damage. To our knowledge there are no human clinical reports available on the accumulation of DNA adducts in ocular inflammatory diseases. Hence the aim of the present study was to determine the levels of 8-hydroxy-2'deoxyguanosine (8-OHdG) and to correlate it with the antioxidant and oxidant parameters in patients with ED and healthy control subjects. METHODS: Twenty-two patients with ED [12 in active perivasculitis (AV) and 10 in healed vasculitis stages (HV)] were recruited for the study. 14 healthy volunteers were included as control. 8-OHdG was determined in their leukocytes by Gas chromatography-Mass Spectrometry (GC/MS) technique. Glutathione levels were determined in plasma, while Superoxide dismutase (SOD) activity and thiobarbituricacid reactive substances (TBARS) content were determined in their erythrocytes. RESULTS: Our results indicated that 8-OHdG levels were elevated by 3 and 1.9 folds respectively in AV and HV stages of patients with ED, when compared with healthy control subjects. Increased 8-OHdG levels correlated with diminished SOD activity and decreased GSH content in patients with ED. CONCLUSIONS: To our knowledge, this is the first report of DNA adduct accumulation in patients with ED. Increased DNA adduct accumulation correlated with the severity of the disease, and they lie in parallel with diminished antioxidant capacity observed in patients with ED. Based on the observations from our present work and our earlier studies, we reiterate that oxidative stress is involved in the disease process. Hence we believe antioxidant vitamin E and C supplementation might be beneficial to patients with ED.
PURPOSE: We examined a patient with acute posterior multifocal placoid pigment epitheliopathy and bilateral central retinal vein occlusion. METHOD: Case report. A 28-year-old woman presented with the typical findings of acute posterior multifocal placoid pigment epitheliopathy. One month after presentation, the patient developed bilateral central retinal vein occlusion. RESULT: Four months after presentation, resolution of the bilateral central retinal vein occlusion resulted in a corrected visual acuity of RE, 20/25 and LE, 3/400. CONCLUSION: Vasculitis, sometimes associated with acute posterior multifocal placoid pigment epitheliopathy, can affect the ciliary vessels that supply the optic disk. This may result in axoplasmic stasis and compression of the central retinal vein, leading to impending or complete central retinal vein occlusion.
Systemic vasculitis is an uncommon manifestation of X-linked lymphoproliferative disease (XLP), a disorder in which there is a selective immune deficiency to Epstein-Barr virus (EBV). The molecular basis for XLP has recently been ascribed to mutations within SLAM-associated protein (SAP), an SH2 domain-containing protein expressed primarily in T cells. The authors describe a patient who died as a result of chronic systemic vasculitis and fulfilled clinical criteria for the diagnosis of XLP. Sequencing of this patient's SAP gene uncovered a novel point mutation affecting the SH2 domain. The patient presented with virus-associated hemophagocytic syndrome (VAHS) and later had chorioretinitis, bronchiectasis, and hypogammaglobulinemia develop. He further developed mononeuritis and fatal respiratory failure. Evidence of widespread small and medium vessel vasculitis was noted at autopsy with involvement of retinal, cerebral, and coronary arteries as well as the segmental vessels of the kidneys, testes, and pancreas. Immunohistochemical analysis using antibodies to CD20, CD45RO, and CD8 revealed that the vessel wall infiltrates consisted primarily of CD8(+) T cells, implying a cytotoxic T-lymphocyte response to antigen. EBV DNA was detected by polymerase chain reaction (PCR) in arterial wall tissue microdissected from infiltrated vessels further suggesting that the CD8(+) T cells were targeting EBV antigens within the endothelium. The authors propose that functional inactivation of the SAP protein can impair the immunologic response to EBV, resulting in systemic vasculitis.
We describe 4 patients with coagulopathy and vasculitis that demonstrated marked tissue ischemia and necrosis. In the clinical setting of rapid evolution of vascular insufficiency, the possibility of combined vasculopathic processes should be considered. This is especially so in patients with underlying connective tissue disease such as systemic lupus erythematosus or rheumatoid arthritis, in which both vasculitis and antiphospholipid antibodies are frequently present. Treatment of both pathologic processes may be required to prevent progressive tissue ischemia and necrosis. The use of antiplatelet and/or anticoagulation should be given consideration in addition to immunosuppressive agents.
BACKGROUND: Our studies had indicated that optic disc vasculitis (ODV) is a distinct clinical entity. We investigated the presentation and clinical characteristics of ODV and determined the efficacy of systemic corticosteroids in its management. METHODS: From 1973 to 1997, we investigated 32 patients (34 eyes) with ODV. The information was obtained by complete medical and ophthalmic history taking and a detailed ophthalmic examination at the initial and follow-up visits. Non-parametric analysis of demographic characteristics and Cox proportional hazard modeling of treatment outcomes was performed. RESULTS: The most common presenting symptom was blurred vision (in 31/34 eyes--91.2%). Visual acuity was 6/12 or better in 82.4% and 6/30 or better in 94.1% of the eyes. All eyes had an enlarged blind spot, vitreous cells and optic disc edema. Fluorescein angiography demonstrated retinal peripapillary phlebitis in 52.6% of the eyes. Systemic hypertension in young patients (P=0.004) and frequency of smoking (P=0.004 for males, P=0.046 for females) were significantly higher than in the general population. Treatment with systemic corticosteroids improved the rate of resolution of optic disc edema and vitreous cells, reducing median resolution times (P=0.042). There was a tendency towards improved central visual fields with treatment (P=0.09). Final visual acuity was 6/12 or better in 25 (92.6%) and 6/18 or better in all of the 27 eyes followed by us. CONCLUSION: Our study indicates that our cases represent a distinct clinical entity of ODV and not just a potpourri of unilateral optic disc edema of different etiologies. It is a self-limiting disease, usually with good prognosis, and benefits from treatment with systemic corticosteroids. The presence of retinal peripapillary phlebitis on fluorescein angiography suggests mild vasculitis of the optic nerve head as the primary pathological mechanism in this disorder.
BACKGROUND: By allowing one to detect fluorescence beyond the retinal pigment epithelium, indocyanine green angiography (ICGA) has made it possible to analyse the choroidal vessels. Our aim was to characterize choroidal vasculitis in posterior uveitis using ICGA. METHODS: Charts of active posterior uveitis patients with a specific diagnosis seen in the different centers participating in the study who had undergone dual fluorescein and ICG angiography were reviewed. The type of inflammatory involvement of the choroidal circulation at entry and the treatment response on follow-up angiograms were analysed. RESULTS: A total of 129 patients were analysed. Choroidal vasculitis could be subdivided into two main patterns: (1) primary inflammatory choriocapillaropathy and (2) stromal inflammatory vasculopathy. The first pattern consisted of hypofluorescent areas up to the late phase of angiography characteristic for choriocapillaris non-perfusion and included entities such as multiple evanescent white dot syndrome (MEWDS), acute posterior multifocal placoid pigment epitheliopathy (APMPPE), multifocal choroiditis (MC), ampiginous choroidopathy and serpiginous choroidopathy. The second pattern consisted of fuzzy indistinct appearance of vessels in the intermediate angiographic phase and diffuse choroidal hyperfluorescence in the late phase indicating inflammatory vasculopathy of larger choroidal vessels. This pattern was found in all cases of active Vogt-Koyanagi-Harada disease, ocular sarcoidosis and tuberculosis and birdshot chorioretinopathy. In Behçet's uveitis of recent onset, choriocapillaris perfusion delay and fuzzy choroidal vessels without diffuse late choroidal hyperfluorescence was found. In posterior scleritis, enlargement of vorticous veins was an additionnal ICGA sign. Stromal inflammatory vasculopathy always responded to anti-inflammatory therapy. A third group of patients with severe retinal or choroidal inflammation presented with associated secondary inflammatory choriocapillaropathy angiographically identical to the primary involvement. CONCLUSIONS: ICGA allowed the hitherto impossible characterization of inflammatory involvement of the choroidal vessels, showing either predominant inflammation of the choriocapillaris or predominant inflammation of the stromal choroidal vessels with or without secondary choriocapillaritis. ICGA will be indispensable for the correct evaluation and follow-up of posterior inflammation with suspected choroidal involvement.
Antineutrophil cytoplasmic antibodies are seen in patients with systemic vasculitides, especially Wegener's granulomatosis. Antineutrophil cytoplasmic antibodies are helpful laboratory markers for these disease. We report on the ocular findings of six patients with systemic vasculitis who had antineutrophil cytoplasmic antibodies. Four patients had systemic Wegener's granulomatosis, one had microscopic polyarteritis, and in one a specific histopathologic diagnosis could not be made. Two patients were first evaluated for systemic vasculitis because of their ocular manifestations. Ocular findings included ptosis, bilateral lacrimal gland masses, proptosis, choroidal folds, episcleritis, phlebitis, retinal and vitreous hemorrhage, keratitis sicca, and bilateral central scotomas. It was difficult to make a systemic diagnosis in all cases. If systemic vasculitis is in the differential diagnosis of a patient with suggestive ocular findings, antineutrophil cytoplasmic antibody testing should be considered. A prospective study of antineutrophil cytoplasmic antibody testing should be considered in patients with ocular findings that suggest the possibility of vasculitis.
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Eales' disease (ED) is a rare condition characterized by repeated retinal and vitreous hemorrhages. The only extraocular involvement described occasionally in the literature is neurological. Histologically, vasculitis in ED is usually restricted to the eye, but occasionally involves the central nervous system, where demyelinizing lesions may also occur. We present a 34-year-old male with ED and subclinical central nervous system involvement. Craneal magnetic resonance images (MR) suggested demyelinization; brainstem auditory and somatosensory evoked potentials were abnormal. There was moderate pleocytosis in CSF and intratecal production of immunoglobulins with oligoclonal bands. Follow-up over a period of 2.5 years showed no clinical, MR or CSF changes in spite of continued opthamological impairment. Little is known about factors that affect the development or not of demyelinizing lesions in ED patients with neurological involvement demonstrated by intratecal production of immunoglobulins. Identification of such factors may contribute to our understanding of other diseases, such as multiple sclerosis.
BACKGROUND: We report on a patient with Graves' disease with radiation retinopathy caused by low-dose irradiation and antithyroid drug-induced antineutrophil cytoplasmic antibody (ANCA)-positive vasculitis. CASE: A 38-year-old woman with Graves' disease presented with bilateral blurred vision, microaneurysms, telangiectasia, and macular edema. The patient was examined by ophthalmoscopy and fluorescein angiography, and radiation retinopathy was diagnosed. OBSERVATIONS: The patient had been treated with low-dose irradiation for her Graves' ophthalmopathy a few years earlier. She also had ANCA-positive vasculitis induced by the antithyroid drug (propylthiouracil, PTU) that had been prescribed for her at that time. Because of multiple avascular areas on both retinas, she was treated by intensive retinal photocoagulation to control progressive retinopathy. CONCLUSIONS: The radiation doses used to treat Graves' disease ophthalmopathy are low. Nevertheless, there is still a risk of radiation retinopathy developing in patients with PTU-induced ANCA-positive vasculitis.
A case of unilateral central vein occlusion occurring in a normotensive patient with systemic lupus erythematosus (SLE) is reported. This vasculitis is related to SLE. In spite of the observation of circulating immune complex and a positive response to the human basophil degranulation test in the presence of bovine soluble retinal antigen, the real pathogenesis of this unilateral vasculitis remains obscure.
During the past 5 years 412 patients (189 male, 223 female) presenting with vascular disorders were examined with Doppler ultrasonography (DUSG) of the orbital vessels. DUSG was used as a screening method for detecting additional lesions of the carotid system. The 412 patients were divided into 5 groups according to their clinical symptoms: Group I: amaurosis fugax; group II: occlusions of the central retinal artery; group III: venous circulatory disorders of the retina; group IV: AION and optic disk vasculitis; group V: symptoms of chronic ocular ischemia. DUSG disclosed a high percentage of additional carotid lesions in groups I and II. In retinal venous disorders, in idiopathic non-arteritic AION and in optic disk vasculitis DUSG findings usually were negative. In contrast to this, DUSG furnished valuable diagnostic information in arteritic AION, where nearly 50% of DUSG findings were positive. Carotid angiography, echo-flow examination of the carotid system and temporal biopsy were used as reference methods.
400 patients presenting with active inflammation of the conjunctiva, sclera, orbita, uvea or retina were cANCA negative during active disease, however, pANCA were found in 15%. Another group of 100 patients suffering from Wegener's granulomatosis (WG) showed in 72 cases one or multiple ocular inflammatory symptoms. They were frequently cANCA-positive. Contrary to some other reports dealing with ocular inflammation, we find a high specificity of cANCA for Wegener's granulomatosis and, therefore, recommend cANCA-testing in all ocular inflammatory disorders, which may be difficult to differentiate from a manifestation of WG.
The Acquired Immunodeficiency Syndrome (AIDS), caused by the human immunodeficiency virus (HIV), also called the human T-lymphotropic virus type III/lymphadenopathy-associated virus [HTLV-III/LAV], has affected over 23,000 people; more than half of those with the disease have died. The actual case fatality rate approaches 100%. AIDS affects all groups and classes of people, although some are at special risk. Distribution of the disease is worldwide. The illness' effects on the body are widespread; of special interest are the ophthalmologic manifestations. The eye may be infected by various viruses (cytomegalovirus, varicella-zoster virus, herpes simplex virus or HIV itself), toxoplasma gondii, candida sp, cryptococcus neoformans, M. tuberculosis, or M. avium-intracellulare. Kaposi's sarcoma may affect the eye as well. Retinal vascular abnormalities (e.g., cotton-wool spots, vasculitis) are not uncommon in AIDS. The syndrome may present with neuro-ophthalmologic manifestations. No effective treatment for the illness is currently available, although several hold promise and there is hope for an AIDS vaccine. Prevention of infection through reduction of risks appears to be the only defense against AIDS at this time.
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