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PubMed2006

[Cotton-wool spots as first ocular manifestation of giant cell arteritis].

INTRODUCTION: Giant cell arteritis is the most common vasculitis in the elderly. Visual loss is established as the most dreaded and irreversible complication of the disease, which makes giant cell arteritis an ophthalmic emergency. It is important to recognize its various ocular manifestations; isolated cotton wool spots are one of them. CASE REPORT: We describe a patient with constitutional symptoms and mild visual loss who was found to have giant cell arteritis. The ophthalmic examination was significant for only isolated cotton-wool spots. A delay in arm-to-retina circulation time was demonstrated by fluorescein angiography. Prompt treatment with corticosteroids led to preservation of vision and uneventful resolution of the cotton-wool spots. DISCUSSION: Common ophthalmologic presentations include anterior ischemic optic neuropathy or choroidal ischemia when short posterior ciliary arteries are involved, and central retinal artery occlusion. Retinal ischemia has also been recognized as a possible manifestation. Because the disease concerns medium-size and larger arteries, it cannot involve terminal retinal arterioles to produce cotton-wool spots. These are probably caused by platelet microembolization from the partially thrombosed regional arteries. CONCLUSION: Isolated cotton-wool spots can be an early ophthalmoscopic finding in giant cell arteritis and precede severe irreversible visual loss. Their detection is essential because it allows the ophthalmologist to establish a prompt treatment and preserve vision.

Diagnosis, Differential↗
PubMed2003

Nonnecrotizing herpetic retinopathies masquerading as severe posterior uveitis.

OBJECTIVE: Aqueous humor analysis can be performed in severe atypical forms of posterior uveitis unresponsive to conventional treatment to exclude a viral infection. DESIGN: Noncomparative interventional case series. PARTICIPANTS: Thirty-seven immunocompetent patients seen with corticosteroid-resistant forms of posterior uveitis underwent extensive evaluation, including anterior chamber paracentesis, to rule out a nonnecrotizing viral retinopathy. INTERVENTION: Aqueous fluid samples were prospectively obtained. Polymerase chain reaction (PCR) and serologic evaluation of intraocular antibody production against herpesviruses were performed by molecular techniques and enzyme-linked immunosorbent assay. MAIN OUTCOME MEASURES: Polymerase chain reaction and local antibody production for herpes simplex virus types 1 and 2, varicella-zoster virus, cytomegalovirus, and Epstein-Barr virus were determined on aqueous fluid samples. RESULTS: Viral infection was confirmed in 5 cases (13.5%). Clinical presentation included birdshot-like retinochoroidopathy, occlusive bilateral vasculitis, and cystoid macular edema. An antiviral regimen was initiated in all cases. Inflammation was stabilized, and steroid dosage could be significantly reduced. CONCLUSIONS: Identification of a viral agent during severe posterior uveitis can dramatically change therapeutic management.

Acyclovir↗
PubMed1996

Takayasu arteritis: clinical characteristics and the role of genetic factors in its pathogenesis.

Takayasu arteritis is a chronic vasculitis, the aetiology of which is still unknown. Several characteristic clinical manifestations have led scientists to consider the possibility of the genetic factors in the aetiology, conjecture which is supported by HLA analysis. Recent attention has focused on the different clinical manifestations among Asian and South American countries and further progress is likely to stem from international network studies.

Adult↗
PubMed2002

Ocular manifestations of hepatitis C virus infection.

Approximately 3.6 million persons in the United States are infected with the hepatitis C virus (HCV), a condition with both hepatic and extrahepatic sequelae. Although no pathognomonic manifestation of HCV infection in the eye has been demonstrated, associations between HCV infection and various ocular syndromes have been reported in small case series and individual patients. At this time, the ocular manifestations of HCV infections best supported by the literature include a dry eye syndrome similar to Sjögren syndrome, and ischemic retinopathy caused by either an HCV-induced vasculitis or treatment with interferon. Patients with diabetes seem to be more susceptible to interferon retinopathy and to subsequent permanent visual loss. There have been no cases of HCV transmission via corneal transplantation, suggesting that current cadaveric screening protocols are effective in preventing this route of transmission. Screening for HCV should be considered in patients with risk factors for HCV infection who suffer from unexplained ischemic retinopathy or dry eyes.

Antiviral Agents↗
PubMed2006

Giant cell arteritis.

Giant cell arteritis (GCA) is an immune-mediated vasculitis, affecting medium- to large-sized arteries, in individuals over the age of 50 years. Visual loss is a frequent complication of GCA, and once it occurs it tends to be both permanent and profound. Although major advances have been made in recent years in genetics, molecular biology and the description of the vessel wall morphology, the aetiology and pathogenesis of GCA are still incompletely understood. Over the years there has been much debate over whether polymyalgia rheumatica and GCA are separate or linked entities. Recent investigations support that polymyalgia rheumatica and GCA are two different expressions of the same underlying vasculitic disorder. A single cause or aetiological agent has not as yet been identified. Except for the histopathology of the arterial wall, there are no laboratory findings specific for GCA, and no particular signs or symptoms specific for the diagnosis. GCA typically causes vasculitis of the extracranial branches of the aorta and spares intracranial vessels. Transmural inflammation of the arteries induces luminal occlusion through intimal hyperplasia. Clinical symptoms reflect end-organ ischaemia. Branches of the external and internal carotid arteries are particularly susceptible. Corticosteroids remain the only proven treatment for GCA, the regimen initially involving high doses followed by a slow taper. However, early detection and treatment with high-dose corticosteroids is effective in preventing visual deterioration in most patients.

Giant Cell Arteritis↗
PubMed1996

Fusarium endophthalmitis in an intravenous drug abuser.

PURPOSE: We studied a case in which a patient had unilateral retinal infiltrates and a retinal vasculopathy resembling frosted branch angiitis. He later admitted to injecting cocaine intravenously. METHODS: The patient underwent a pars plana vitrectomy and received intravitreal and intravenous amphotericin B. RESULTS: The vitreous fluid grew Fusarium dimerium. There was rapid response to the treatment and full recovery of vision. CONCLUSION: Fusarium species should be considered as a potential pathogen in intravenous drug abusers with endogenous endophthalmitis and in patients with unilateral frosted branch angiitis.

Adult↗
PubMed1984

[Neuroretinitis].

Eight patients presenting with the characteristic clinical picture of neuroretinitis are described. The decrease in visual acuity was caused by a papillitis, whereas the macular star developed later in the course of the disease when the visual acuity had already recovered. Five of our 8 cases were adults, a fact which demonstrates that neuroretinitis is not only confined to childhood. Three of the 8 cases were bilateral, two of them children. Two patients presented with neuroretinitis and additional inflammatory processes of other ocular segments (one with chorioretinitis and vasculitis; one with scleritis and uveitis), suggesting that a general vascular inflammation may be present in neuroretinitis. The macular star is produced by lipid-rich exudate in Henle's nerve fiber layer, thus giving the picture of stellate retinopathy. The exudate probably leaks from the disk due to a defect in the blood-tissue barrier or a loosening of the border tissue at the disk margin. The etiology of neuroretinitis remains an enigma. Serologic and cultural tests for preceding viral infections have so far been inconclusive.

Adolescent↗
PubMed1977

The ocular manifestations of Wegener's granulomatosis. Fifteen years experience and review of the literature.

Ocular manifestations of Wegener's granulomatosis may occur secondary to contiguous granulomatous sinusitis or as a result of focal vasculitis. Contiguous granulomatous sinus disease causes nasolacrimal duct obstruction, proptosis and ocular muscle or optic nerve involvement. Focal vasculitis unrelated to contiguous upper respiratory tract disease is manifested by conjunctivitis, episcleritis, scleritis, corneoscleral ulceration, uveitis, and granulomatous vasculitis of the retina and optic nerve. A review of 29 cases of Wegener's granulomatosis and three cases of lymphomatoid granulomatosis studied over the past 15 years at the National Institute of Allergy and Infectious Diseases (NIAID) disclosed single or multiple ocular manifestations of disease in 15 patients (47 per cent). The pattern of ocular disease, its relationship to systemic involvement, diagnostic methods and the response to therapy are discussed.

Adolescent↗
PubMedDate not supplied

Intra-arterial BCNU chemotherapy for the treatment of malignant gliomas of the central nervous system: a preliminary report.

We treated six patients with unilateral malignant astrocytomas with pulse doses of intra-arterial BCNU (200 mg/m2 initially, with escalating doses every 6-8 weeks) via transfemoral selective internal carotid catheterization. Four patients had had partial resections without prior radiation therapy and received no steroids. They had decreased tumor stain and surrounding edema after two to four cycles of chemotherapy. For one patient, results of the neurologic examination returned to normal, with total disappearance of her tumor as assessed by computerized tomographic scan. Objective tumor response continued for 7 months in three patients and for 3 months in one. In two of the four patients, chemotherapy was discontinued because of retinal toxicity and not because of treatment failure. Two patients had had partial resections and radiation therapy. One patient had stable disease for 4 months, and the second had progressive disease with gradual visual loss beginning in the infused eye 3 weeks after the second treatment. The catheterization procedure is safe; it was without immediate complication in 17 BCNU infusions. In summary, high-dose intra-arterial BCNU is well-tolerated and is an effective initial chemotherapeutic modality.

Adult↗
PubMed1991

[Postoperative infections in systemic immunocompromising diseases. A study of 6 patients].

Advances of therapeutic strategies in the management of critically ill patients have resulted in an increased life expectancy and more frequent presentations to ophthalmic surgeons. Six patients with postoperative endophthalmitis after elective intraocular surgery suffering from Sharp-syndrome, metastasizing antrum carcinoma, granulomatous vasculitis and immunosuppressive therapy, agranulocytosis, silico-tuberculosis and chronic lymphatic leukemia revealed traditionally pathogenic agents (Proteus mirabilis, P. aeruginosa, enterococci, coagulase-negative Staph). Two patients had to be enucleated, one turned blind and three had a visual acuity 5/100 to 20/60. In our hands prognosis for successful therapy in immunodeficient patients seems worse than in otherwise healthy persons. Painstaking evaluation of history, medical work-up, conjunctival swabs and application of bactericidal topical antibiotics preoperatively and perioperatively are particularly important in these patients.

Aged↗
PubMed1994

Indocyanine green video angiographic findings in acute posterior multifocal placoid pigment epitheliopathy.

The placoid lesions of acute posterior multifocal placoid pigment epitheliopathy consist of two distinct areas: a light yellow area surrounding a dark yellow center. On two occasions, the authors compared indocyanine green video angiographic with fluorescein angiographic findings in a typical case of acute posterior multifocal placoid pigment epitheliopathy. In the acute stage, the multiple placoid lesions observed in the early phase of indocyanine green video-angiography showed hypofluorescent lesions corresponding to those seen with fluorescein angiography which blocked choroidal vessels. Some fluorescent intermediate sized choroidal vessels, which traversed the hypofluorescent areas, were dark due to a slight degree of blocked fluorescence. On both indocyanine green video and fluorescein angiography, hypofluorescent lesions were almost the same size as the light yellow area and the dark yellow center of a placoid lesion. A hypofluorescent lesion, seen on fluorescein angiography, obscured a retinal vessel. When the placoid lesions had disappeared, fewer hypofluorescent lesions, corresponding to some of the hypofluorescent lesions originally observed, were seen in the early phase of indocyanine green video angiography. They were also smaller in size. These hypofluorescent lesions decreased in size and some had completely disappeared in the late phase. No hypofluorescent lesions were seen in the early phase of fluorescein angiography. Findings in the acute stage suggest that the hypofluorescence observed in the early phase of indocyanine green video and fluorescein angiography is mainly blockage due to a light yellow area of placoid. When the placoid lesion disappeared, filling delays, which were detectable on only indocyanine green video angiography, persisted in the choriocapillaris.

Acute Disease↗
PubMed2004

Review of severe vaso-occlusive retinopathy in systemic lupus erythematosus and the antiphospholipid syndrome: associations, visual outcomes, complications and treatment.

PURPOSE: To discuss the pathogenesis of severe vaso-occlusive retinopathy in systemic lupus erythematosus (SLE), the association with antiphospholipid antibodies, and its implications for management and prognosis. METHODS: An illustrative case history of a woman with SLE and severe vaso-occlusive retinopathy in the presence of antiphospholipid antibodies is presented. A literature review of previously reported cases and previously published data on the topic was performed and forms the basis for discussion. RESULTS: This is a rare form of retinopathy in SLE as distinct from the more common, benign form, being classically a microangiopathy with diffuse capillary non-perfusion and small arterial or arteriolar occlusions in the retina. Poor visual outcomes with visual loss are reported in 80% of cases with neovascularization occurring in 40% of cases. It is associated with antiphospholipid antibodies, typically characterized by microthrombosis and immune complex mediated vasculopathy rather than a true vasculitis. There is a strong association between this severe form of retinopathy and central nervous system manifestations of SLE. Anticoagulation has a role in the secondary prevention of thrombosis in the presence of antiphospholipid antibodies, but the role of aspirin and immunosuppression is unclear in the treatment of this condition. Vigilant ophthalmic follow up and aggressive treatment of neovascularization and vitreous haemorrhage can prevent further visual loss. These points are highlighted in the brief case report presented. CONCLUSION: Severe vaso-occlusive retinopathy is a rare form of retinopathy in SLE often associated with poor visual prognosis and neovascularization. It may be a manifestation of the antiphospholipid syndrome. Treatment is aimed at preventing further thrombosis and complications arising from neovascularization.

Adult↗
PubMed1997

[Posterior scleritis in Wegener disease].

BACKGROUND: Wegener's granulomatosis is a focal necrotizing granulomatous vasculitis of unknown etiology. It manifests initially as localized inflammation, usually of the lungs, and preceeds to the state of generalisation which can be life-threatening due to renal failure. Ocular and orbital involvement occurs in 28-58% of patients with Wegener's granulomatosis. More often, the anterior segments of the eye are involved whereas inflammation of the posterior segments is quite uncommon. PATIENTS AND METHODS: We report a case of posterior scleritis as the initial manifestation of Wegener's granulomatosis in a 65-year-old woman. RESULTS: Posterior scleritis responded favourably to therapy with systemic steroids and cyclophosphamide. CONCLUSIONS: Wegener's granulomatosis can have a poor prognosis if not diagnosed and treated in time. Knowledge of posterior scleritis as a possible presenting symptom may therefore facilitate early diagnosis and treatment and prevent progression of the disease.

Aged↗
PubMed2004

[Clinical study on treatment of childhood Henoch-Schonlein purpura nephritis with colquhounia root tablet].

OBJECTIVE: To study the effect of Colquhounia root tablet (CRT) in treating childhood Henoch-Schonlein purpura nephritis (HSPN) and compared with tripterygium wilfordii multiglycoside tablet (TWMGT). METHODS: Eighty-two children with HSPN were divided into the CRT group and the control group. To patients of nephritic type, treatment with CRT and TWMGT was applied separately, but to those of nephrotic type, the treatment was given together with prednisone. Therapeutic effect and changes of related indexes, including urinary protein, retinal-binding protein (RBP), N-acetyl-beta-glucosaminidase (NAG), were observed after patients received treatment for 6 months. RESULTS: The complete remission (CR) rate and partial remission (PR) rate in patients of nephritic type in the treated group was 58.8% and 41.2% respectively, while those in the control group was 20.8% and 66.7% respectively, and the ineffective rate being 12.5%. Comparison of the therapeutic effect between the two groups showed significant difference (P < 0.05). Although the CR rate in patients of nephrotic type in the treated group was higher than that in the control group, the difference was insignificant (P > 0.05). The urinary levels of protein, RBP and NAG lowered in both groups, but level of urinary protein in the treated group of nephritic type after 6 months of treatment was significantly lower than that in the control group (P < 0.05). CONCLUSION: CRT shows obvious effect in improving childhood HSPN of nephritic type, nephrotic type and attenuating the tubulointerstitial lesions, compared with TWMGT, CRT is more effective in lowering proteinuria and better in total effective rate.

Child↗
PubMedDate not supplied

[Clinical aspects of the nephropathy in Schoenlein-Henoch syndrome].

Fourty-four children with Henoch-Schoenlein nephritis were studied at the onset of the nephropathy and during a follow-up from 6 to 110 months. The extra-renal manifestations were purpura (100%), abdominal pain (63.5)% or melena (27%), arthlagias (61.5%), neurological symptoms with convulsions (4.5%) and retinal involvement (4.5%). The clinical presentation of the nephropathy consisted in haematuria and proteinuria (41%), isolated haematuria (30%), acute renal failure (ARF) (23%), nephrotic syndrome (4%) or isolated proteinuria (2%). Hypertension was present in 17 patients. Renal biopsy was performed in 18 patients and the glomerular changes were graded according to the classification of ISKDC; the renal histopathology ranged from minimal lesions to severe crescentic glomerulonephritis and was found to correlate with clinical state. Twenty-four patients, who showed severe clinical presentation and/or diffuse mesangial proliferation with high proportion of crescents, received a corticosteroid therapy. Most of our patients followed a relatively benign course: all but one of patients with ARF have normal renal function at the end of follow-up and no patients with less severe renal presentation got a bad outcome. Only 2 patients showed relapse of nephropathy and purpura at the 6th and 8th year of follow-up, respectively. After 24 months of follow-up the clinical outcome of a group of 19 patients receiving corticosteroid therapy was not very different from that of 11 untreated patients.

Acute Kidney Injury↗
PubMed2001

Multiple arterial ectasias in patients with sarcoidosis and uveitis.

PURPOSE: To describe and evaluate the cause of a clinical entity characterized by bilateral intraocular inflammation, multiple arterial ectasias including beading, macroaneurysms, comma-like ectasias and kinking, with vasculitis, staining of the optic disk and multiple peripheral round punched-out hypopigmented chorioretinal scars in elderly patients. The formation and the course of the arterial ectasias is analyzed. METHODS: Seven patients with this syndrome were evaluated by clinical examination, fluorescein angiography, and systemic investigations. RESULTS: Three of the seven patients had a biopsy characteristic of sarcoidosis, two others showed positive bronchoalveolar lavage, as well as other analyses and tests suggesting sarcoidosis, and two showed other findings suggestive of sarcoidosis. The patients were all over 60 years of age and had arterial hypertension. In two patients, an arterial ectasia developed at the site of previous focal inflammation. The macroaneurysms either remained unchanged, became comma-like ectasias, arterial kinks, or completely vanished. CONCLUSION: Elderly patients with multiple arterial ectasias, uveitis, disk staining, and peripheral chorioretinitis should be thoroughly investigated for sarcoidosis. We suggest that sarcoidosis may cause some degree of arteritis, which may weaken the arterial wall, with resulting ectasia. Arterial hypertension may play a role in the formation of the ectasias by increasing the pressure on the arterial wall weakened by inflammation.

Adult↗
PubMed1997

[Retinochoroidal involvement of type II membranoproliferative glomerulonephritis. An angiographic study with indocyanine green].

PURPOSE: Type II measangiocapillary glomerulonephritis is related to dense deposits within the glomerular basal membrane and the basal membrane of the pigment epithelium (Bruch's membrane). Being a vasculitis, an angiographic study by indocyanine green (ICG) could possibly enlarge the semiologic features of this disease. METHODS: The indocyanine green angiographic changes in 3 patients with predialitic renal failure due to type II measangiocapillary glomerulonephritis (MCGN II) (dense deposit disease) are reported. A complete ophthalmologic examination, electroretinography, electro-oculography, fluorescein and indocyanine green angiography (ICG) were performed for each patient. RESULTS: Abnormal hyperfluorescent dots were seen on the same part of the fundus on both fluorescein and ICG angiography, though these locations were different for each of the three patients. These results seem to link the deposits to vascular changes within the choriocapillaris, which opposes them to drusen encountered in age related macular degeneration (ARMD). It appears that the choriocapillary lesions could be similar to the glomerular disease. CONCLUSION: Beyond the diagnostic challenges related to the nephrologic disease, it is known that subretinal neovascularization occurs in some cases of MCGN II, although the pathophysiologic mechanism of the deposits is probably not the same as in ARMD. Therefore, ICG angiography should be performed when MCGN II is first known, serving as an initial examination for further follow-up.

Adult↗
PubMed1987

[Deep venous thrombosis in Behçet's disease. 106 localizations in a series of 177 patients].

A retrospective study of 177 patients (137 male, 40 female) with a disease that fulfilled the criteria of complete or incomplete Behçet's disease showed 106 thrombotic lesions of a venous trunk in 62 (35%) of these patients, superficial phlebitis and retinal vein thrombosis excluded. The incidence of thrombosis was unrelated to race and sex. The venous lesions were single in 38 cases and multiple in 24 cases, including 4 with more than 4 territories involved. Arterial lesions were also present in 7 cases. The lesions were located in the sural (n = 63), iliofemoral (n = 14), inferior vena cava (n = 13), superior vena cava (n = 3), axillary (n = 2) and cerebral (n = 13) veins or territories. Thrombosis developed during the first 4 years of Behçet's disease in one half of the patients. It preceded the aphthosis by 1 to 10 years in 11 cases, was concomitant with it in 15 cases and appeared more than 10 years after the diagnosis in 8 cases. Pulmonary embolism occurred in 11 patients, requiring clipping of the vena cava in 2, but it was never lethal. However, 2 of the 5 patients who died had venous thrombosis (Hughes Stovin syndrome). Thirty-eight patients could be followed up for 12 to 192 months (mean 43) under anticoagulant or antiplatelet therapy. Five vascular recurrences (13%) were observed, 2 of them (1 arterial graft thrombosis, 1 cerebral thrombosis) under anti-vitamin K agents. Cerebral thrombosis (9 men, 4 women) accounted for 27% of the neurological lesions; its prognosis was favourable in the 7 patients successfully treated with anticoagulants. Thrombosis could not be explained. Haemostasis studies showed a decrease of fibrinolytic activity compared to a group of normal subjects and an increase in fibrinogen, factor VIII and Willebrand's factor levels; these abnormalities did not seem to be specific. Thus, phlebitis truly is an element of Behçet's disease. Until its mechanism is better understood, effective anticoagulant therapy in cases with proven thrombosis and preventive antiplatelet therapy in the other cases seem to be rational measures.

Adult↗