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pANCA antibodies in patients with anterior uveitis: identification of a marker antibody usually associated with ulcerative colitis.

A pANCA autoantibody (antineutrophil cytoplasmic antibody, perinuclear pattern) has been described in uveitis patients, but its correlation with systemic illnesses and the specific type of pANCA has not been defined. The goals of this study were to determine the (1) frequency of pANCA autoantibodies in uveitis, (2) systemic associations in the pANCA + uveitis patients, and (3) type of pANCA antigen recognized by the uveitis-associated autoantibody. Serum was obtained from 59 patients with anterior uveitis or panuveitis and from nonuveitis controls. A detailed medical and family history was obtained from each subject at the time of phlebotomy. Sera were screened by neutrophil ELISA to determine the frequency of ANCA positivity. Immunofluorescence assays were then used to differentiate cANCA from pANCA. The specificity of the pANCA + antibodies was further characterized by DNase 1 sensitivity and granule antigen ELISAs. ANCA antibodies were detected in 29% of all patients with panuveitis or anterior uveitis. In 41% of these ANCA + patients, serum antibody detected a perinuclear antigen that was sensitive in all cases to DNase 1. The majority of pANCA + uveitis patients were either HLA-B27 positive or had systemic evidence of immune-mediated diseases. Two pANCA + patients had no medical or family history of other immune-mediated diseases. This study identifies a subset of uveitis patients distinguished by expression of a specific pANCA marker antibody. The characteristics of this antibody are similar to the pANCA antibody present in most patients with ulcerative colitis. Expression of the pANCA autoantibody in uveitis patients is a susceptibility marker for other immune-mediated diseases.

Adult↗

Vitreous surgery in the management of chronic endogenous posterior uveitis.

OBJECTIVES: There is evidence that pars plana vitrectomy (PPV) has a beneficial effect on the clinical course of chronic endogenous posterior uveitis (EPU) possibly by physically removing any resident inflammatory cells with the vitreous. We assessed the anatomical and therapeutic effects of PPV performed on patients with chronic EPU for any indication. PATIENTS AND METHODS: Retrospective review of 41 eyes of 38 consecutive patients with EPU who underwent a PPV for any reason, over a 5-year period. The mean age of the patients was 36.2 years, 46% of the eyes had intermediate uveitis, 32% panuveitis, and 22% posterior uveitis. The visual acuity, disease activity, and the requirement for medications to control it were recorded for 12 months pre- and postoperatively. RESULTS: Overall, 61% of the eyes gained more than 2 Snellen lines (P<0.001) and the incidence of cystoid macular oedema (CMO) significantly reduced from 44 to 20% (P<0.05). Postoperatively, there was a significant decrease in the recurrence rate of intermediate uveitis, posterior uveitis, and panuveitis (P<0.001). The use of systemic and local depot immunosuppressive agents did not change over the study period, although the use of topical agents increased (P<0.05). CONCLUSION: PPV appears to have a beneficial effect on the clinical course of EPU in selected cases. This may be mediated by the physical clearance of inflammatory debris, the anti-inflammatory effect of replacing vitreous by aqueous humour, by a reduction of CMO and/or the anatomical correction of sight-threatening retinal pathology.

Adolescent↗

[Endogenous fungal endophthalmitis: results of antifungal treatment with and without vitrectomy].

BACKGROUND: Fungal endophthalmitis represents a significant cause of ocular morbidity, affecting in the majority of cases patients in poor general conditions. The eye is typically involved by hematogen dissemination, and the germ induces a chorioretinitis associated with an important panuveitis. PATIENTS AND METHODS: Four patients were examined. They complained of a progressive reduction of vision, associated with photophobia. Ophthalmologic examination disclosed an important panuveitis. Investigations showed a fungal chorio-retinitis in all cases. Risk factors were intra-venous toxicomania, longterm parenteral nutrition and traumatism of the sinuses. RESULTS: Vitreous cultures were positive for Candida albicans (3 patients) and for Aspergillus fumigatus (1 patient). Blood cultures were negative in the four cases. Three patients were treated with anti-fungal medication (fluconazole, itraconazole) associated with a vitrectomy by pars plana. One patient was treated by anti-fungal therapy only. Clinical evolution was satisfactory in all cases. Final vision was 10/10 in three cases and 5/10 in one. One patient developed a retinal detachment and an epiretinal membrane. Follow-up was 7 months (2-16 months). CONCLUSION: The evolution of these four cases suggests that a rapid anti-fungal therapy associated with or without a vitrectomy represent a favourable therapeutic option when a fungal infection is suspected.

Adult↗

[Ocular involvement in Whipple disease].

PATIENTS AND METHODS We present a 57-year-old white man with progressive deterioration of vision, who had a prolonged history of weakness, migratory arthralgias and loss of weight. Bilateral panuveitis, with iritis, inflammatory vitreous opacities associated with small, round, grayish retinal lesions. The duodenum showed a swollen mucosa, which was flecked over with pinpoint grayish grains. A small-bowel biopsy disclosed PAS-positive granules in the macrophages of the lamina propria mucosa, pathognomonic of Whipple's disease. RESULTS After antibiotic treatment with trimethoprim and sulfamethoxazole there was no relapse of the panuveitis during the follow-up period of 18 months.

Humans↗

Leptospirosis.

Leptospirosis, a waterborne spirochetal illness, is common in tropical climates. Rodents and wild animals are the most common reservoirs for this widespread zoonosis. Human disease is acquired by contact with urine or tissues of an infected animal or through contaminated water and soil. Systemic leptospirosis is characterized by its multisystem involvement, protean manifestation, and varying severity. The clinical presentation ranges from occult infection to fatal complications like hepatorenal failure. Early diagnosis is important to halt the fulminate course. The microagglutination test is considered the gold standard serological test for leptospirosis, although molecular techniques are now under study. Treatment of systemic leptospirosis includes penicillin or tetracycline in addition to supportive treatment. Ocular involvement occurs during the immunological phase of the disease. One or both eyes may be involved, typically with a panuveitis often accompanied by retinal periphlebitis and hypopyon. In general, leptospiral uveitis has a good prognosis and the patient recovers full vision in spite of severe panuveal inflammation. Awareness of this infectious uveitic entity is essential not only in order to differentiate it from other severe autoimmune uveitides, but also to reduce the percentage of idiopathic uveitis in endemic areas.

Eye Diseases↗

Cutaneous larva migrans, sacroileitis, and optic neuritis caused by an unidentified organism acquired in Thailand.

We report the case of a 32-year-old pregnant woman with an unidentified intraocular parasite. The parasite, which had been acquired in Thailand, caused cutaneous larva migrans, sacroileitis, and 2 years later optic neuritis and panuveitis. The patient was successfully treated with ivermectin and albendazole. The diagnostic possibilities of this peculiar presentation are discussed. Parasitic infections are a leading cause of medical problems in travelers to tropical countries.1 While most parasites cause gastrointestinal problems, some may migrate throughout the body and lodge in critical organs. Ocular parasitic infections may occur by direct inoculation onto the eye,2 or incidentally during systemic migration. Subconjunctival parasites are easily diagnosed by removal and careful microscopic examination.3 Parasites, which lodge within the eye, are more difficult to diagnose, especially if not removed. In this report we describe a patient who presented with an intraocular parasite causing optic neuritis and panuveitis, 2 years after travel to Thailand.

Adult↗

Pediatric uveitis in southern Turkey.

Uveitis in the pediatric population is not so common, and the etiology is different from adults. In the present study the charts of the patients with onset of uveitis at 16 years of age or younger were reviewed in order to analyze the etiology in pediatric patients with uveitis, and to compare the results with other studies carried out on different populations. The charts of 90 cases, followed at the Uvea-Immunology Clinic of the Cukurova University Medical Faculty Department of Ophthalmology, between January 1987 and February 1996 were reviewed retrospectively. There were 47 girls, 43 boys, aged 5-20 (13.64 +/- 4.24) in the study group. The average age at onset of uveitis was 12.20 +/- 4.81, and the follow-up period was 9-98 months (21.80 +/- 11.13). Of the 90 patients 31 (34.4%) had panuveitis, 30 (33.3%) had anterior uveitis, 21 (23.3%) had posterior uveitis and 8 (8.9%) had intermediate uveitis. Only 2 (9.5%) of the patients with posterior uveitis were considered idiopathic after extensive laboratory and clinical work-up, whereas idiopathic cases constituted 48.4% of panuveitis, and 46.7% of anterior uveitis cases. Of the 90 patients an associated condition could be found in only 59 (65.6%) patients. Of these 59 patients 23 had toxoplasmosis (39%), which constituted the most common associated condition in this study. The second most common underlying cause was Behçet's disease (17%), followed by pars planitis (13.6%), Fuchs' heterochromic iridocyclitis (8.5%), JRA (5.1%), leukemia (5.1%), and herpetic eye disease (5.1%). There were single cases with Reiter, toxocariasis, traumatic uveitis, and sympathetic ophthalmia. Environmental, cultural and genetic factors may be accountable for the differences in relative frequencies of some of the associated conditions between our findings and those of previously published studies in patients with pediatric uveitis.

Adolescent↗

[Evaluation and monitoring of selected inflammation patterns in uveitis using laser tyndallometry].

AIM: The purpose of the present study was to evaluate the degree of inflammation and to monitor the dynamics of the blood-aqueous barrier disruption in selected cases of uveitis using laser tyndalometry. MATERIAL AND METHOD: Measurements with the use of laser tyndalometer (Kowa FM-500) were performed in 72 patients (90 eyes) with various types of uveitis. They were divided into four groups: anterior uveitis (28 eyes), intermediate uveitis (pars planitis) (28 eyes), posterior uveitis (26 eyes) and panuveitis (8 eyes). Aqueous flare values were expressed as photon counts per millisecond. RESULTS: Tyndalometric mean values in control eyes were 4.8 +/- 1.0 ph/msec. Mean initial flare was pronounced in multifocal choroiditis and panuveitis--196.0 ph/msec, HLA-B27 positive acute anterior uveitis--145.4 ph/msec, and in acute herpes zoster anterior uveitis--52.4 ph/msec. It was mild to moderate in Fuchs uveitis syndrome--7.8 ph/msec, pars planitis--15.7 ph/msec, posterior uveitis in toxoplasmosis--6.8 ph/msec and toxocariasis--17.5 ph/msec. The potential of laser flare-meter for precise follow-up and adjustment of therapy was demonstrated in selected cases. CONCLUSIONS: Laser tyndalometry has been proved to be a useful tool for the objective and quantitative evaluation of anterior chamber flare in uveitis and for monitoring the effectiveness of the treatment, thus improving therapeutic efficacy of uveitis.

Adolescent↗

[Childhood Behçet's disease: clinical and evolutive aspects. About 13 cases].

INTRODUCTION: Behçet's disease is a systemic vasculitis which rarely occurs in childhood. The aim of this study was to evaluate clinical characteristics and outcome of Behçet's disease in Moroccan children. MATERIAL AND METHODS: A retrospective study of 13 cases of children, 10 males and 3 females with Behçet's disease followed up between 1990 and 1998. The diagnosis of Behçet's disease was based on the criteria of the international study group for Behçet's disease. All patients were studied by a complete clinical, ophthalmological and laboratory staging and treated with appropriate therapy. RESULTS: The mean age at diagnosis of Behçet's disease was 13.9 years. Familial forms were found in 30.7% of cases. Oral aphtae were noted in all cases while genital ulcers were present in 76% of cases. Cutaneous lesions were found in only 1 case and 53.8% of children had a pathergy test. Articular involvement was found in 30.7%, neurological features in 46% and vascular manifestations in 38.4%. Only one case of intestinal involvement was noted. Ocular features (76%) were bilateral in all cases and were dominated by panuveitis complicated by macular edema ant retinal vasculitis. CONCLUSION: Behçet's disease seems to have particular characteristics in childhood. Familial forms, articular and digestive manifestations appear to be more frequent in early stages of Behçet's disease in children. Neurological and vascular involvement with panuveitis seems more frequent in the older children.

Adolescent↗

[Cyclosporin A in therapy of chronic uveitis].

48 patients with chronic severe uveitis of pressumed noninfectious origin resistent to corticosteroids have been treated with Cyclosporin A. All patients had an initial loading dose of 5 mg/kg/day followed by a dose reduction according to ocular inflammatory activity and tolerability and according the guidelines by BenEzra, Nussenblatt and Timonen. Most of the patients received additional corticosteroids in a low dose. 35 out of these 48 patients (13 suffering from intermediate uveitis, 11 from retinal vasculitis, 5 panuveitis, 4 "pressumed histoplasmosis" and 2 sympathetic ophthalmia) were treated for 1 year and observed after withdrawing of Sandimmun for at least 6 months. The majority of these patients have manifested a positive therapeutic response to Cyclosporine, in particular patients suffering from vasculitis, panuveitis and sympathetic ophthalmia. All 35 patients were treated before for a long time with steroids without success, 19 out of these 35 in addition with cytotoxic agents. The immunosuppressive effect of Cyclosporine was not permanent, frequently the inflammation relapsed on reduction of dosage or withdrawing of the drug. Guidelines for combined regimen (Cyclosporine and corticosteroids and vitrectomy) were given. Although a large variety of side effects were reported the compliance was good.

Administration, Oral↗

[The serology of toxoplasmosis in uveitis].

Determinations for decelerating the antitoxoplasma anticorps that have been effectuated for 696 uveas proved positivity at 123 aerums (17.6%). The repartition of the positive serology concerning the clinic form of the uvea has proved 48 iridocyclitis, 26 serous central corioretinals, 23 in panuveitis, 13 in posterior uveitis, 11 muscular chorioretinitis, 2 in hyalitis. Taking into consideration only the equal or less than 1:160 titrures, these have been at 7 iridocyclitis, 4 serous central chorioretinitis, 4 panuveitis, 3 posterior uveitis and 3 atrophic central chorioretinitis. It is shown that the diagnosis of ocular toxoplasma must be effectuated just corroborating the clinic data with the positive serology. The antiparasitic treatment doesn't influence upon the low titrures of serous anticorps, which generally maintain at the same value all the life.

Animals↗

Clinical manifestations of Behçet's disease in 137 Italian patients: results of a multicenter study.

OBJECTIVE: To determine the type and frequency of clinical features of Behçet's disease in a population of Italian patients. METHODS: We retrospectively studied 137 Italian patients (76 males and 61 females, age at onset 29.6 +/- 12.2 [mean +/- SD] years) seen consecutively in nine different referral centers. The duration of follow-up at study entry was 10.9 +/- 8.2 years. Virtually all patients fulfilled the classification criteria developed by the International Study Group for Behçet's disease. The clinical manifestations of the patients were recorded by the attending physicians using specifically designed forms. RESULTS: The most frequent manifestations at disease onset were oral (78.3%) and genital aphthae (29.2%) followed by inflammatory ocular involvement (20%) and arthritis (14.2%). The commonest (>50% of cases) manifestations observed throughout the disease course were oral aphthae (99.3%), genital aphthae (62.8%), various cutaneous lesions including erythema nodosum (81.8%), and inflammatory ocular disease (60.6%). Panuveitis and posterior uveitis/retinitis occurred more frequently in males compared with females (28.9% versus 11.5% and 57.9% versus 36.1%, respectively; p < 0.05). 61.6% of our patients were HLA-B51 positive. CONCLUSION: Behçet's disease in Italian patients is characterized by a variety of clinical manifestations in agreement with the medical literature. Panuveitis and posterior uveitis/retinitis occur more frequently in male patients.

Adult↗

[Vogt-Koyanagi-Harada disease].

BACKGROUND: Vogt-Koyanagi-Harada disease is a bilateral panuveitis associated with exudative retinal detachment. This disease typically affects young adults, and occurs most frequently among Asians. Meningeal signs, dysacusis, poliosis and vitiligo are usual features of the condition. MATERIAL AND METHODS: We present three patients with Vogt-Koyanagi-Harada disease. RESULTS: All patients presented with decreased vision. Two of them had typical prodromal symptoms, with headache and meningism. Bilateral panuveitis with exudative retinal detachment and choroidal effusion developed in all patients. Fluorescein angiography demonstrated areas of pinpoint hyperfluorescence at the level of the pigment epithelium and pooling of dye in the area of exudative detachments. All patients responded well to high-dose systemic corticosteroids, with resolution of the exudative retinal detachments and improved visual acuity. Interpretation. Vogt-Koyanagi-Harada disease is a rare, but important, diagnosis in patients with bilateral uveitis. Early administration of high-dose systemic steroids is recommended for successful treatment.

Adolescent↗

[Value of alveolar lavage and transbronchial biopsy in the diagnosis of isolated sarcoid uveitis].

The diagnosis of sarcoidosis is established by biopsy of sarcoid tissues demonstrating non-caseating granuloma, but difficulties arise when extra pulmonary organs are involved separately. Positive histologic signs can however be found in the lung even when there are no radiologic features. Bronchoalveolar lavage and biopsies were performed in 19 patients presenting severe, strictly isolated uveitis (5 cases of anterior uveitis, 3 cases of posterior uveitis and 11 cases of panuveitis). Criteria of positivity were non-caseating granuloma and lymphocytosis. Positive signs were obtained in 6 cases (31,5%); 1 case of posterior uveitis and 5 of panuveitis. There was no case of false positive results but one false negative result. There was a lack of correlation between results of these investigations and angiotensin converting enzyme blood levels. These investigations are non-invasive in experienced hands.

Adult↗

["Primary" reticulum-cellsarcoma of the retina. I. Clinico-pathologic study of 5 patients (author's transl)].

Between 1964 and 1974 a primary reticulum-cellsarcoma of the retina was diagnosed histologically in 5 patients (between 44 and 71 years), in one already clinically. The initial diagnosis had been "uveitis" (2), "panuveitis" (1), "iridocyclitis with central retinal artery occlusion" (1), and "chorioretinitis" (1). The usual antiinflammatory therapy was without effect in every instance. All patients showed neurological symptoms with cerebral manifestations. Twice the cerebral biopsies had been misinterpreted initially as "atypical glioblastoma multiforme", once as Neuro-Behçet. The disease progressed over a course of 2--10 years from the initial ophthalmic symptoms to death. A review of the literature is given and the differential-diagnosis to necrotizing forms of retinitis, dissiminated chorioiditis neoplastic processes of retina and uvea and degenerative diseases are discussed. The primary reticulum-cellsarcoma of the retina must be considered in the differential-diagnosis of uveitis or panuveitis if 1. there is progression in spite of the usual antiinflammatory therapy, 2. the initial infiltrations are seen in the deep layers of the sensory retina.

Adult↗

So-called 'acute retinal necrosis syndrome'--an acute ocular panvasculitis syndrome.

An acute ocular disorder, characterized by panuveitis, retinal vasculitis, retinal necrosis (with frequent secondary retinal detachment) and frequent optic atrophy, has been reported since 1971 under different eponyms in 74 patients (103 eyes). I report 4 more cases. This paper presents, based on a critical review of 107 eyes in 78 patients, a detailed clinical description, pathogenesis and terminology of this distinct ocular disease entity. The available clinical and histopathological evidence very strongly indicates that the basic pathology is acute vasculitis of the iris, choroid, retina and optic nerve head, producing vascular occlusion in the choroid, retina and optic nerve head; the clinical findings thus represent acute ischemic lesions of those tissues, in addition to panuveitis. From the available evidence, it seems more appropriate to call this condition 'acute ocular panvasculitis syndrome' rather than 'acute retinal necrosis syndrome' since retinal necrosis is only one of a number of lesions seen in this disease. The etiology of the panvasculitis still remains a mystery.

Acute Disease↗

Ocular histopathologic findings in a case of human herpes B virus infection.

A 37-year-old male laboratory technician who sustained a cutaneous penetrating wound from a rhesus monkey developed a progressive ascending encephalomyelitis due to culture-proven herpes B virus (Herpesvirus simiae) infection. He died 6 weeks after his injury despite acyclovir and ganciclovir treatment that was initiated after central nervous system symptoms developed. Histopathological examination of the patient's left eye revealed a multifocal necrotizing retinitis associated with a vitritis, optic neuritis, and prominent panuveitis. Herpes-type virus was identified in the involved retina by electron microscopy. Postmortem vitreous cultures taken from both eyes and retinal cultures taken from the right eye were positive for herpes B virus. Herpes B virus produces infection and destruction of retinal tissues similar to other herpesviruses. To our knowledge, this case represents the first histopathologic demonstration of herpes B virus infection in a human eye.

Adult↗

Tuberculin skin-test-induced uveitis in the absence of tuberculosis.

A 16-year-old Caucasian girl who showed no evidence of tuberculosis or other systemic disease developed acute panuveitis progressing to bilateral serous retinal detachments following purified protein derivative (PPD) skin testing on two separate occasions separated by an interval of 8 years. Both episodes responded rapidly to steroid therapy. Uveal hypersensitivity to PPD skin testing has been described in the presence of ocular or systemic tuberculosis, Eales' disease, and the Vogt-Koyanagi-Harada syndrome (VKH). Our case, however, suggests that profound uveitis may be precipitated by routine PPD skin testing alone.

Adolescent↗