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PubMed2002

High dose intravenous steroid therapy for severe posterior segment uveitis in Behçet's disease.

AIM: To evaluate the safety and effectiveness of high dose intravenous steroid therapy (HDIST) in Behçet's disease patients with severe posterior segment uveitis attacks. METHODS: Five patients with ocular Behçet's disease were treated with HDIST for severe posterior segment attacks. Two patients had vasculitis, one patient had papillitis, and the other two had retinitis; four patients also had accompanying severe vitritis. The visual acuities and improvement in ocular signs after HDIST were evaluated. RESULTS: During HDIST, patients had no systemic complications caused by treatment. All patients responded to HDIST with evidence of a decrease in intraocular inflammatory activity and improved visual acuities within a mean time of 7 (1-15) days of commencing treatment. During follow up three out of five patients had new posterior segment attacks. CONCLUSION: HDIST is effective in controlling severe, vision threatening acute posterior segment Behçet's uveitis attacks and in improving visual function in a short period of time

Administration, Oral↗
PubMed1999

Pathologic findings in a steroid-responsive optic nerve infarct in giant-cell arteritis.

OBJECTIVE: To investigate the pathophysiologic mechanism of optic nerve infarction in giant-cell arteritis (GCA). BACKGROUND: Previous pathologic reports of optic nerve infarction in GCA involved patients who were blind at the time of death. The optic nerve infarcts were primarily retrolaminar in localization. Simultaneous short ciliary and ophthalmic artery vasculitis was found in all patients. METHODS: Clinical neurologic and ophthalmologic examination, temporal artery biopsy, and neuroimaging tests were performed in a patient with an anterior ischemic optic neuropathy secondary to GCA. Pathologic examination of the viscera, eye, and brain were performed at autopsy 1 month later. RESULTS: A prelaminar/retrolaminar infarct was found in this patient. Subsiding vasculitis was limited to the short ciliary arteries, sparing the central retinal, pial, and ophthalmic arteries. CONCLUSIONS: The authors believe that the visual improvement observed in this patient was the result of preserved, anterior optic nerve collateral circulation, as well as the neuroprotective and anti-inflammatory effect of the corticosteroids.

Aged↗
PubMed1984

Natural course of nonaphakic cystoid macular edema.

We evaluated the longterm natural history of nonaphakic cystoid macular edema (CME) in a retrospective study of 130 out of 557 CME cases recorded in the past ten years. A listing of causes was provided and the cases divided into two groups: those with perifoveal leakage and those with deep subretinal leakage. In cases of diabetic retinopathy, 60 patients who were followed up for more than three years had noncystoid or cystoid macular edema. The occurrence and persistence of a large central foveal cyst usually resulted in a severe decrease in visual acuity. Hard exudates, present in 60% of cases, seemed to influence visual prognosis when they were inside the foveal avascular zone. In cases of venous occlusion, chronic CME increased the risk of a central cyst and was the major cause of a macular scar. In cases of uveitis and vasculitis, the restoration of macular capillary wall competence was possible when inflammation decreased. Disturbances in the macular pigment epithelium were also shown to produce poor visual acuity.

Choroid Neoplasms↗
PubMed2003

Cerebral vasculitis in a patient with HCV-related type II mixed cryoglobulinemia.

The peripheral nervous system is often involved in patients with mixed cryoglobulinemia (MC), while there are few reports of central nervous system involvement. We describe a case of HCV-related type II MC with peripheral and central nervous system involvement. A 61-year-old woman, suffering from flaccid tetraparesis, was referred to our department because of an increasing disability. The presence of delirium prompted us to also investigate the central nervous system. MMSE, EEG, EMG, brain CT-scan, color-Doppler of neck vessels, retinal fluorangiography and brain MRI were performed. These investigations suggested a cerebral vasculitis. The finding of very low C4 serum levels, together with high rheumatoid factor serum levels, suggested the search for cryoglobulins. The laboratory findings showed a HCV-related type II (IgMk) MC. A marked improvement of symptoms and of laboratory data was obtained by treatment with methylprednisolone + cyclophosphamide.

Cryoglobulinemia↗
PubMed1999

Intraocular-central nervous system lymphoma: clinical features, diagnosis, and outcomes.

OBJECTIVE: To analyze the clinical features, laboratory investigations, and diagnosis of intraocular-central nervous system (CNS) lymphoma in a cohort of patients who underwent diagnostic vitrectomy. DESIGN: Retrospective case series. METHOD AND STUDY MATERIALS: Thirty-four vitreous biopsy specimens obtained from 26 patients with treatment-resistant or unusual uveitis were re-evaluated in a masked fashion. The specimens were classified into three groups: "negative," "suspicious of malignancy," and "positive" based on the cytologic features, immunomarkers, and flow cytometry. The medical records of the patients were reviewed retrospectively. MAIN OUTCOME MEASURES: The reliability of vitreous cytology in diagnosing intraocular-CNS lymphoma and the differences in clinical features of patients with intraocular-CNS lymphoma and uveitis. RESULTS: The two ocular pathologists concurred in their criteria for interpretation of all specimens. There was 100% concordance between the cytologic reports read independently by the two ocular pathologists over the 5-year period and the read-out done in a masked fashion at the time of the study. Ten patients were diagnosed with intraocular-CNS lymphoma based on the vitreous cytology and clinical features. The time interval between the initial presentation and vitreous biopsy was 1 week to 2 years, with 80% of the patients diagnosed within the first year. Retinal involvement in the form of lymphomatous subretinal pigment epithelial infiltrates, vasculitis, and apparent retinochoroiditis was present in six cases. Initial neuroimaging studies revealed concomitant CNS involvement in three patients, and an additional three developed CNS lymphoma following diagnosis by vitreous biopsy. Patients were treated with radiotherapy, chemotherapy, or both. Two of the four patients with a follow-up of greater than 12 months died due to CNS involvement. CONCLUSIONS: Vitreous cytology is a sensitive, reliable, and reproducible method of diagnosing intraocular-CNS lymphoma. A high index of suspicion based on the clinical findings and course of the uveitis is critically important in decision-making for diagnostic vitrectomy. Central nervous system involvement is frequent and associated with a high mortality rate. Ophthalmology 1999;106:1805-1810

Adult↗
PubMed2005

[Temporal arterious obliteration by Candida endophthalmitis].

We report the case of a 50-year-old woman with a branch retinal artery occlusion caused by acute Candida albicans chorioretinitis, diagnosed based on ophthalmoscopic and angiographic examinations. Although vasculitis is frequent during Candida endophthalmitis, branch obstruction is rare. The thrombosis is probably related to perivascular inflammation rather than septic embolus. We propose antiaggregant therapy when the infection is near the retinal vessels.

Candidiasis↗
PubMed1999

Serum hepatocyte growth factor as a possible indicator of vascular lesions.

To investigate the role of human hepatocyte growth factor (hHGF) in vascular lesions associated with endothelial injury, we measured serum hHGF concentrations in subjects with retinal arteriosclerosis, coronary atherosclerosis, or arteriolitis due to Henoch-Schönlein purpura. Individuals with more advanced grades of retinal arteriosclerosis showed higher serum hHGF concentrations [grade 0, 0.053+/-0.005 ng/mL (n = 68); grade 1, 0.144+/-0.022 ng/mL (n = 21; P<0.01 vs. grade 0); grade 2, 0.338+/-0.036 ng/mL (n = 20; P<0.01 vs. grade 0 or 1); grade 3, 0.526+/-0.051 ng/mL (n = 9; P<0.01 vs. grade 0, 1, or 2)]. Patients with active arteriolitis due to Henoch-Schönlein purpura showed higher (P<0.01) serum hHGF concentrations (0.347+/-0.038 ng/mL; n = 14) than those in the remission phase (0.097+/-0.017 ng/mL; n = 19). Mean serum hHGF concentrations were higher in subjects with coronary atherosclerosis than in those without, but a significant overlap in serum hHGF concentrations was found between subjects with and those without coronary atherosclerosis. Serum hHGF may be an indicator of the presence or development of arteriolar lesions.

Aged↗
PubMed1994

Cytomegalovirus vasculitis. Case reports and review of the literature.

Cytomegalovirus (CMV) infection is a substantial cause of morbidity and mortality among immunocompromised patients. It may present with a mild, self-limited syndrome, retinitis, colitis, or invasive disease with pneumonitis, hepatitis, and bone marrow suppression. We review another, less common manifestation of CMV disease: CMV-associated vasculitis. CMV may productively infect vascular endothelial cells (25), causing a local vasculitis (3, 14, 19) and ischemia. Alternatively, the host immune response to cells expressing viral antigen may be the stimulus for vasculitis (12, 53). Since there are no pathognomonic appearances to mucosal or cutaneous lesions, biopsy of accessible sites is critical for diagnosis and expeditious initiation of appropriate antiviral therapy. The CMV-associated vasculitides represent a broad spectrum of diseases, with GI vasculitis in nontransplant recipients having the best prognosis. Cutaneous vasculitis associated with CMV seems to be a more fulminant disease, with the majority of cases having a fatal outcome. These differences likely reflect the degree of viral burden and the state of immune competence. Additionally, since the virus itself is immunosuppressive, host defenses may be further compromised by the infection. Although a large collective experience assessing the impact of ganciclovir and foscarnet is not currently available, both the prompt initiation of antiviral treatment and a concurrent reduction in any immunosuppressive regimen, including steroids, should be undertaken since these therapeutic strategies have clearly improved outcome for other CMV syndromes (22, 34, 55). As the number of recipients rises and the HIV pandemic spreads we are likely to see an increase in the number of cases of vasculitis associated with CMV infection.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗
PubMedDate not supplied

Susac syndrome in four male patients.

PURPOSE: To report the clinical and imaging features in four male patients presenting with Susac syndrome, a microangiopathy affecting the brain, the retina, and the cochlea. METHODS: Retrospective review of clinical data, fluorescein angiograms, and magnetic resonance imaging findings in these four cases. RESULTS: All four patients were young men (range, 20-35 years). The axiomatic triad of ocular, cochlear, and neurologic involvement was present in three patients. Neurologic symptoms were absent in the fourth one. Fluorescein angiography showed arteriolar wall hyperfluorescence in all four patients. Magnetic resonance images showed in three patients multifocal hyperintense lesions in the white matter and the corpus callosum with typical involvement of the central fibers. Therapeutic modalities and clinical course are described. Three patients had a follow-up of 3, 5, and 13 years with complete remission of the disease within 1 year in all three cases. One patient had severe neuropsychological sequelae. CONCLUSION: Susac syndrome seems to be less unusual in men than previously reported. Though presenting as a self-limited monophasic course disease in most cases, it may result in severe neuropsychological sequelae. Early diagnosis of the syndrome is enabled by the combination of the ophthalmologic, audiometric, and brain magnetic resonance features.

Adult↗
PubMed2000

Raised platelet thiobarbituric acid-reacting substances in proliferative Eales' disease.

BACKGROUND: Platelets are an elective site for oxidative stress owing to their high content of polyunsaturated fatty acid. Increased lipid peroxidation and elevated platelet thiobarbituric acid-reacting substances (TBARS) signal oxidative stress. This possibly leads to retinal neovascularization in Eales' disease. METHODS: TBARS levels were estimated in consecutive cases of Eales' disease with neovascularisation (n = 26), Eales' disease without neovascularisation (n = 17) and healthy controls (n = 17). RESULTS: Platelet TBARS levels in the cases of Eales' disease with neovascularisation, Eales' disease without neovascularisation, and healthy controls were 0.66 +/- 0.1, 0.57 +/- 0.11 and 0.42 +/- 0.14 n moles TBARS formed/hour/10(8) platelets respectively. Student's t-test showed a significant increase in platelet TBARS levels in cases with neovascularisation as compared to cases without neovascularization (p < 0.05) and healthy controls (p < 0.01). CONCLUSION: The increase in platelet TBARS levels in proliferative Eales' disease is consistent with an emerging view that lipid peroxides may be associated with retinal neovascularisation.

Adolescent↗
PubMedDate not supplied

Ocular manifestations of leptospirosis.

Leptospiral uveitis is a common entity in tropical countries. Ocular manifestations are noted in the second phase of illness, but these remain under-diagnosed mainly because of the prolonged symptom-free period that separates the systemic manifestations from detection of ocular manifestations. Varying ophthalmic presentations and the intrinsic nature of different types of uveitis to mimic one another also challenge the accuracy of the diagnosis. Of the individual ocular signs, the combination of acute, non-granulomatous, panuveitis, hypopyon, vasculitis, optic disc edema, membranous vitreous opacities and absence of choroiditis or retinitis have high predictive value for the clinical diagnosis of leptospiral uveitis. Geographic location of the patient, occupation, socio-economic status, risk factors related to exposure, past history of fever or jaundice also aid in diagnosis. Steroids are the mainstay of treatment for leptospiral uveitis. Depending upon the severity and anatomical location of inflammatory lesion, topical, peri-ocular and/or systemic steroids are given. The prognosis is generally good, even when the inflammation is severe.

Eye Diseases↗
PubMed1996

[Ophthalmologic complications due to human immunodeficiency virus infection at Bamako (Mali)].

AIDS ocular complications have been researched in 70 hospitalised patients in the two main hospitals of Bamako (Mali) during one year (1992-1993). Men were predominant (sex ratio 1.6). HIV1 infections (67%) were most frequent than HIV1 + HIV2 (21.4%) or HIV2 infections (11.4%). Most of the patients were on the WHO's clinical stage III; 34% of them had ocular complications, quite often non infectious: cotonous nodules (10%), vascularitis (5.7%) and retineous haemorrhages (4.3%). Ocular opportunistic infections were rare: only one case of toxoplasmic chorio-retinitis was reported. Ocular complications were observed with all types of HIV. Vascular abnormalities were observed in the stage II or IV of AIDS and seemed, in Bamako, as a serious sign during the AIDS course.

Adolescent↗
PubMed1986

Ten cases of optic vasculitis.

Optic disc vasculitis is an idiopathic, usually benign condition in young healthy adults. Hayreh suggested that the condition is to be divided into two types: type 1, with optic disc edema and a benign course as the dominant features and type 2, a condition resembling a central retinal vein (CRV) occlusion with a variable outcome depending on the extent and the site of the lesion. Differential diagnosis with other causes of one-sided papilledema (type 1) and other vascular conditions (type 2) is very difficult but important. We suggest performing extensive ophthalmological and neurological examinations to exclude major diseases. In this article we will give a review of the literature and a description of the ten patients with optic disc vasculitis who were seen in our clinic between January 1983 and August 1986.

Adult↗
PubMed1995

Elevated anti-neutrophil cytoplasmic antibody titer in a patient with atypical orbital pseudotumor.

We recently examined a patient with ophthalmic manifestations of Wegener's granulomatosis in whom antineutrophil cytoplasmic antibody (cANCA) titers provided helpful diagnostic information. A 40-year-old man who had suffered from bilateral exophthalmos for 9 months was diagnosed initially as having idiopathic inflammatory pseudotumor. The patient exhibited purulent nasal discharge and microhematuria. A histopathologic study revealed vasculitis. His ANCA titer for cANCA was found to be evaluated, and our patient was subsequently diagnosed as having Wegener's granulomatosis. His ocular symptoms resolved and did not recur after treatment with corticosteroid in combination with cyclophosphamide. We believe that cANCA levels should be investigated in patients with orbital pseudotumor as a possible sign of Wegener's granulomatosis.

Adult↗
PubMed1985

A microangiopathic syndrome of encephalopathy, hearing loss, and retinal arteriolar occlusions.

A syndrome consisting of a subacute encephalopathy, sensorineural hearing loss, and retinal arteriolar occlusions is described in two women. Laboratory investigations did not reveal any systemic vasculitis. CT and cerebral angiography showed no abnormalities, but magnetic resonance imaging revealed small, discrete lesions in the white matter. Biopsy of cortical brain from one patient showed disseminated microinfarcts in the gray matter as well as sclerosis of small vessels. This syndrome is characterized as an occlusive vasculopathy rather than vasculitis, and should be considered in evaluations of young women presenting with encephalopathy and hearing loss.

Adult↗