Papillophlebitis, optic disc vasculitis.
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Adult↗
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Results for “Retinal Vasculitis”
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Vasculopathies affecting the eye.
The retinal arteries and veins may be involved in isolation or as the result of a systemic vasculitis. This article emphasizes neurologic diseases in which the ocular vasculature is affected.
Eye↗
Susac's syndrome: beneficial effects of corticosteroid therapy in a Japanese case.
Susac's syndrome is a rare disorder characterized by the triad of microangiopathy of the brain and retina with hearing loss. More than 50 affected individuals have been reported worldwide, all Caucasians. We herein identify the first Japanese patient with Susac's syndrome. A 36-year-old man developed recurrent subacute encephalopathy, bi- a lateral sensorineural hearing loss, and retinal arteriolar occlusions, caused by microangiopathy from a year previously. T2-weighted MRI showed multiple high-signal lesions ti predominantly in the periventricular white matter. During the exacerbated phase both high-dose intravenous methyl-prednisolone and oral prednisone therapy produced beneficial effects. He showed definite remission within 2 years from the disease onset.
Adult↗
Safety of oral methotrexate pulsed therapy.
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Administration, Oral↗
[Uveitis in acquired syphilis].
Syphilis is a well-established cause of uveal disease and is thought to be responsible for several distinct types of intraocular inflammation. Four cases of acquired syphilitic uveitis are reported. The diagnosis was essentially based on the clinical picture, the elimination of other possible causes, the results of reliable blood immunologic tests eg, the TPHA, FTA and Nelson tests, and the rapid response to penicillin therapy. Evaluation of anti-Treponema pallidum antibodies in the aqueous humor does not contribute to the diagnosis of syphilitic uveitis. Two cases needed large amounts of penicillin (total dosage, 440 million units). Oral steroids were used to control the associated inflammatory reaction and to prevent a Herxheimer reaction. All the patients responded well to therapy.
Adolescent↗
[Infrequent manifestations of Henoch-Schonlein purpura].
We review the cases of 148 children with Henoch-Schönlein purpura diagnosed in a period of seventeen years (from 1975 to 1992). Thirteen cases (8.7%) of unusual clinical manifestations were found: testicular affection in six cases (7.6% of the males); IgA nephropathy in four (2.7%); exudative enteropathy in two (1.3%); duodenal syndrome in two; previous abdominal manifestation which led to surgical procedures in one (0.7%) and another case with detachment of the retina. These clinical findings are analyzed and the possibility of unnecessary therapeutic acts is discussed.
Child↗
Susac syndrome: microangiopathy of the retina, cochlea and brain.
BACKGROUND: Susac syndrome is characterized by the triad of branch retinal arterial occlusions, encephalopathy and cochlear microangiopathy. The underlying process is believed to be a small vessel vasculitis causing microinfarcts in the retina, brain and cochlea. METHODS: Analysis of two male and two female cases of Susac syndrome recognized in Australia. RESULTS: In this series the epidemiology, mode of presentation, ophthalmologic features, neurologic and cochleo-vestibular features, radiologic characteristics, cerebrospinal fluid findings, therapeutic interventions, clinical course and outcome of Susac syndrome is examined. Key ophthalmologic differential diagnoses include systemic lupus erythematosis (SLE), Behçet's syndrome and other vasculitides such as sarcoidosis, tuberculosis, syphilis and lymphoma. Neuro-otologic features are most frequently misdiagnosed as multiple sclerosis. CONCLUSION: Susac syndrome, first described in 1979, is becoming an increasingly recognized condition. Early recognition of the syndrome is important because treatment with systemic immunosuppression may minimize permanent cognitive, audiologic and visual sequelae.
Adult↗
Autoimmune ocular disease in MRL/Mp-lpr/lpr mice is suppressed by anti-CD4 antibody.
MRL/Mp-lpr/lpr(MRL/lpr) mice spontaneously have a systemic autoimmune disease, characterized by vasculitis, lymphadenopathy, glomerulonephritis, and autoantibody formation. Among the many autoimmune lesions present are focal ocular inflammatory infiltrates, involving the choroid and sclera. These lesions appear to be related to the vasculitis seen in MRL/lpr mice and are mediated by L3T4-positive helper T-cells (CD4-positive T-cells). Systemic treatment of MRL/lpr mice with a monoclonal anti-L3T4 antibody (anti-CD4) resulted in a dramatic reduction of both the frequency and severity of the ocular disease, supporting the hypothesis that the CD4-positive T-cells play an essential role in the pathogenesis of the choroiditis and scleritis in this strain.
Animals↗
Bilateral retinal ischemia in Kawasaki disease. Postmortem findings and electron microscopic observations.
A 4-month-old infant died of cardiovascular complications of Kawasaki disease (KD). At autopsy, aneurysmal dilatation with recent thromboses were found in coronary arteries, abdominal aorta, brachial and left common carotid arteries. A vasculitis with thrombosis of a branch of the ophthalmic artery was demonstrated in the right orbital apex. Light microscopy disclosed that the ocular abnormalities were confined to the retinas of both eyes that exhibited inner retinal ischemia. By electron microscopy, the inner retina showed clumping of nuclear chromatin, mitochondrial swelling, rupture of plasma membranes, and increased number of lipid figures. Müller cell processes were intact. Stereologic analysis of the mitochondria in the retinal pigment epithelium, photoreceptor inner segment, and ganglion cells in the KD infant demonstrated that identical mitochondrial changes occurred in the retina from an adult woman who had a recent central retinal artery occlusion.
Adult↗
Bilateral simultaneous central retinal artery occlusions in atherosclerosis.
An 82-year-old woman with atherosclerosis developed central retinal artery occlusions (CRAOs) in both eyes within an interval of 5 minutes. There was no evidence of a thromboembolic source, systemic hypotension, vasculitis, migraine, or hypercoagulable state. Virtually simultaneous binocular CRAOs have been rarely reported, and suggest the possibility of a systemic but still undefined "hypercoagulable trigger."
Aged↗
Transient vessel wall sheathing in acute retinal vein occlusions.
Three cases are reported which had features similar to, and evolved in a pattern consistent with central retinal vein occlusions and a fourth case is reported which behaved as a hemispheric vein occlusion. However, they differed from classic retinal vein occlusions by having prominent sheathing of the retinal venous vasculature at presentation, which in all four cases resolved within three weeks. There was no evidence for any of these cases having an inflammatory vasculitis. The significance of this transient sheathing is uncertain.
Acute Disease↗
[Surgical treatment with dacryocystitis and retinal detachment in a patient with Wegener granulomatosis].
BACKGROUND: We report surgical treatment of a patient with dacryocystitis and retinal detachment (RD), which are rare ophthalmic involvements of Wegener granulomatosis (WG). CASE: The patient was a 26-year-old man with WG. He was diagnosed as having WG 4 years ago and he has been treated by maintenance doses of predonisolone and cyclophosphamide. Rheumatoid factor and serum antinuclear antibody were negative. Cytoplasmic pattern-antineutrophil cytoplasmic antibody (C-ANCA) and renal function were normal. He was found to have nasolacrimal duct obstruction and lattice degeneration bilaterally, retinal tear with RD in the left eye and tear without RD in the right eye. No sign of vasculitis was found in fluorescein angiography. Bilateral dacryocystorhinostomy was performed without any sign of postoperative necrosis of the wound. After the surgery, epiphora and eye discharge disappeared and lacrimal passage has been maintained without obstruction. The pathological findings of his nasal mucosa and lacrimal sac showed chronic inflammation and no typical changes of WG. There was no abnormal change in the conjunctiva and sclera after an uncomplicated scleral buckling surgery. CONCLUSION: We conclude that operations such as dacryocystorhinostomy and scleral buckling surgery may be performed successfully when WG is controlled within the normal limits of C-ANCA.
Adult↗
A difficult case of pyrexia.
We present a case of non-specific vasculitis presenting as a pyrexia of unknown origin to illustrate the diagnostic difficulty which can arise with this condition. The diagnostic event was central retinal artery occlusion and the disease was also associated with pyoderma gangrenosum. History, examination, investigations and management are discussed.
Aged↗
Current concepts in the etiology and treatment of Behçet disease.
Behçet disease is an idiopathic, multisystem, chronic, and recurrent disease characterized by exacerbations alternating with phases of quiescence, episodic panuveitis, and aggressive non-granulomatous occlusive vasculitis of the arteries and veins of any size with explosive ocular inflammatory attacks that primarily affect the retinal and anterior segment vasculature of the eye. The disease is characterized by endothelial dysfunction and may produce a wide variety of symptoms. In mild cases, orogenital ulcers and skin lesions are the only findings during the entire clinical course, whereas ocular lesions that occur in more than half of the cases, frequently bilateral, can eventually lead to blindness. Pulmonary, gastrointestinal, and central nervous system involvements may sometimes be life-threatening. This review examines the epidemiology, frequency, immunology, and immunohistopathology of Behçet disease with recent theories of several agents, including phosphoantigens, superantigens, heat-shock proteins, and adenosine deaminase. Perspectives on the possible roles of new etiopathological molecules, such as nitric oxide, endothelin, and homocysteine, are presented. Ocular and systemic clinical features, diagnostic criteria, classifications, laboratory, fundus fluorescein angiography, and radiologic imaging are discussed. Differential diagnosis, disease in pregnancy and childhood, and prognosis with regard to Behçet-induced systemic and ocular complications are also evaluated. Traditional and current treatments with topical, paraocular and systemic corticosteroids, colchicine, dapsone, cyclosporine, azathioprine, methotrexate, cyclophosphamide and chlorambucil are summarized and recent insights into the pharmacology and effects of thalidomide, tacrolimus (FK-506), interferon-alpha, anti-TNF-alpha blocking monoclonal autoantibody (infliximab) and soluble TNF receptor (etanercept) are reviewed. Key clinical investigations with the status of ongoing clinical trials aimed at addressing the drug's efficacy, surgical care, and studies that have raised the possibility of new therapeutic uses are also presented. The challenges posed by the drug's teratogenicity and adverse effects are also considered, if present.
Behcet Syndrome↗
Ophthalmic manifestation of congenital protein C deficiency.
Under normal conditions activated protein C is a natural anticoagulant that cleaves 2 activated coagulation factors, factor Va and factor VIIIa, thereby inhibiting the conversion of factor X to factor Xa and of prothrombin to thrombin. Additionally, activated protein C enhances tissue-plasminogen activator-mediated fibrinolysis by inhibition of plasminogen activator inhibitor-1. This results in an increase in circulatory plasminogen activator levels. Protein C deficiency, a genetic or acquired thrombophilic abnormality, has been demonstrated to predispose to episodes of potentially blinding and lethal thromboembolic events. Heterozygous-deficient subjects usually remain asymptomatic until adolescence or adulthood. In homozygous-deficient patients, protein C activity is usually less than 1% (reference range, 70%-140%), resulting in thromboembolism as early as in the neonatal period. The major clinical symptoms in affected newborn infants have been purpura fulminans, vitreous hemorrhage, and central nervous system thrombosis. The age of onset of the first symptoms has ranged from a few hours to 2 weeks after birth, usually after an uncomplicated full-term pregnancy and delivery. In contrast to the genetic form, acquired neonatal protein C deficiency occurs particularly in ill preterm babies. Typical complications of prematurity such as respiratory distress syndrome, necrotizing enterocolitis, and neonatal sepsis may also be present. In the medical literature, there are only a few reports of homozygous protein C deficiency in neonates. We present 2 cases of homozygous protein C deficiency with ocular and extraocular manifestation.
Anticoagulants↗
Serum homocysteine level is increased and correlated with endothelin-1 and nitric oxide in Behçet's disease.
BACKGROUND/AIMS: Behçet's disease (BD) is a systemic inflammatory vasculitis of young adults with unknown aetiology, characterised by endothelial dysfunction and occlusion in both deep venous and retinal circulation. Ocular involvement occurs in 70% of cases and is characterised by periphlebitis, periarteritis, vascular occlusion, and thrombosis leading to blindness despite vigorous treatment. Endothelin-1 (ET-1) is a vasoconstricting peptide while nitric oxide (NO) is a relaxing molecule and both are released by endothelium for blood flow regulation. Homocysteinaemia is a newly defined term connected to the increased risk of atherothrombotic and atherosclerotic systemic and retinal vascular occlusive diseases, and its role in the course of BD has not been previously described. The authors aimed to detect serum total homocysteine (tHcy), ET-1, and NO in BD and to assess if tHcy, ET-1, and NO are associated with ocular BD or disease activity. METHODS: 43 consecutive patients with ocular (n = 27) or non-ocular (n = 16) BD (36.95 (SD 9.80) years, 22 male, 21 female) satisfying international criteria, and 25 age and sex matched healthy control subjects (37.88 (8.73) years, 13 male, 12 female) without a history of systemic or retinal venous thrombosis were included in this study. Patients were examined by two ophthalmologists with an interest in BD. Serum tHcy, ET-1, and NO concentrations were measured in both groups. Hyperhomocysteinaemia was defined as a tHcy level above the 95th percentile in the control group. Patients were divided into active and inactive period by acute phase reactants including alpha(1) antitrypsin, alpha(2) macroglobulin, erythrocyte sedimentation rate, and neutrophil count. RESULTS: The overall mean serum tHcy, ET-1, and NO levels were significantly higher in patients with BD than in control subjects (tHcy = 15.83 (4.44) v 7.96 (2.66) ng/ml, p <0.001; ET-1 = 17.47 (4.33) v 5.74 (2.34) micromol/ml, p <0.001; NO = 37.60 (10.31) v 27.08 (7.76) micromol/l, p <0.001). Serum tHcy, ET-1, and NO levels were significantly higher in active patients than in inactive patients and control subjects. In addition, among patients with ocular BD, the mean tHcy levels were significantly increased and correlated with ET-1 and NO levels when compared with non-ocular disease and control subjects. All acute phase reactant levels were significantly higher in active period than in inactive stage and controls. CONCLUSIONS: Elevated tHcy may be responsible for the endothelial damage in BD and may be an additional risk factor for the development of retinal vascular occlusive disease, contributing to the poor visual outcome in these patients. Assessment of tHcy may be important in the investigation and management of patients with BD, especially with ocular disease.
Acute-Phase Proteins↗
Ophthalmologic manifestation of Behçet's disease.
Loss of vision in Behçet's disease is one of the most common, as well as one of the most serious, of its varied manifestations. Total blindness may often be the eventual outcome. The underlying problem in the eye, as well as in other organ systems, is an occlusive vasculitis. Although the most common ocular symptom is that of iridocyclitis, the presence of retinovascular lesions, especially necrotizing retinitis, is well known but often obscured by the severity of the anterior reaction, which precludes a good view of the retina. While Behçet's disease is characterized by spontaneous remissions and relapses, no external factors have been found to explain the length of remissions. Blindness, which occurs in up to 25% of patients, is one of the major causes of permanent disability.
Behcet Syndrome↗
[Perioperative management of a patient with purpura fulminans syndrome due to protein C deficiency].
PURPOSE: Protein C is a vitamin K-dependent anticoagulant and homozygous protein C deficiency is a rare fatal thrombotic disease. This report describes the perioperative management of homozygous protein C deficiency in a patient who underwent a total of three surgical procedures under general anesthesia and the successful use of activated protein C concentrate. CLINICAL FEATURES: A female baby, who developed disseminated intravascular coagulation and purpura fulminans shortly after birth, was diagnosed as purpura fulminans syndrome due to homozygous protein C deficiency. At one month of age, she suffered bilateral retinal detachment and glaucoma due to retinal hemorrhage. After marked improvement of her condition after administration of activated protein C concentrate, she underwent a left iridectomy and implantation of a Broviak catheter under general anesthesia. Her intraoperative course was uncomplicated but, on postoperative day four, she presented another episode of massive cutaneous necrosis and gangrene. Activated protein C concentrate was administered again, with good results. She underwent replacement of a Broviak catheter at four months of age, and right iridectomy for glaucoma at eight months. Both were uneventful. CONCLUSION: The perioperative management of homozygous protein C deficiency and purpura fulminans requires appropriate measures for thromboembolic prophylaxis. Sufficient iv fluid administration is necessary. Attention should be paid to decrease the risk of tissue compression such as that associated with positioning, blood pressure cuff, and endotracheal intubation, which may cause necrosis over pressure points. Replacement therapy with activated protein C concentrate appears safe and effective. The anesthetic management is reviewed and discussed.
Anesthesia, General↗