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[Unusual course of Candida endocarditis].

Fever, anemia, pulmonary infiltrates and weight loss occurred in a 47-year-old man four weeks after a gastric operation. On echocardiography and cardiac catheterization an atrial myxoma was revealed. In removing the myxoma the tricuspid valve apparatus was destroyed, requiring insertion of a bio-prosthetic valve (Hancock). Signs of infection persisted post-operatively, together with complete A-V block and panuveitis. The Candida IGM titer was markedly increased and histological examination of the thrombus revealed massive fungal infection. Recurrent fever and massive vegetation on the prosthetic valve necessitated removal of the prosthetic valve under antimycotic treatment, which also cleared the pulmonary lesions. The patient's general condition improved. Right-heart failure was controllable by drugs.

Antifungal Agents↗

Behçet's disease in Western Switzerland: epidemiology and analysis of ocular involvement.

PURPOSE: To determine the epidemiology of Behçet's disease in Western Switzerland and to study the characteristics of ocular disease. METHODS: Patients newly diagnosed with Behçet's disease, seen from January 1990 to December 1998 at Hôpital Jules Gonin, La Source Eye Center, and the Division of Immunology and Allergy, University of Lausanne, were analyzed. The Japanese diagnostic criteria for Behçet's disease were used and both complete and incomplete forms were included. Incidence, proportion of cases with ocular involvement, and the characteristics of ocular disease were determined. RESULTS: During the follow-up period, a total of 35 Behçet's patients were seen in the three study centers. Twenty-five of these patients had ocular involvement. The incidences of Behçet's disease and Behçet's ocular disease in the western part of Switzerland, with a referral population of roughly 600000 persons, were 0.65 and 0.46 per 100000 per year, respectively. The mean age of the group was 33.2 years, age at presentation was 25.8 years, and mean follow-up was 5.7 years. The most frequent sign was oral aphthous involvement (86%), followed by ocular manifestations (72%). The HLA-B51 antigen was present in 66% of the patients with ocular involvement. Panuveitis was present in 20/25 patients (80%), anterior uveitis in 3/25 patients, and posterior involvement in 2/25 patients. Twenty-two patients (88%) had systemic treatment that always included corticosteroids; it was associated with other treatments in 17/22 patients. Mean duration of treatment was 45.5 +/- 25.1 (SD) months. The mean recurrence-free period in the six patients in whom treatment could be interrupted was 18.0 +/- 8.8 (SD) months. Visual function was maintained at the end of the follow-up for the collective and visual outcome was correlated to visual function at presentation. CONCLUSIONS: The incidence of Behçet's disease in Western Switzerland was comparable to that in other western nonendemic countries. With early diagnosis and appropriate therapy, the prognosis was favorable in our collective of patients.

Adrenal Cortex Hormones↗

Incidence of sympathetic ophthalmia after penetrating eye injury and choice of treatment.

INTRODUCTION: Sympathetic ophthalmia, a diffuse bilateral granulomatous panuveitis, is a serious potential complication after penetrating eye injury. Many surgeons recommend enucleation within two weeks of trauma to prevent this condition. We retrospectively reviewed the medical records of patients with penetrating ocular injuries who had either evisceration or enucleation to determine the incidence of sympathetic ophthalmia and to evaluate the surgical interventions and their complications. Age at surgery, time after penetrating trauma, surgery technique, follow-up period, ophthalmic examination of the fellow eye, and surgical complications were evaluated. RESULTS: In total, 217 patients were included in the study with a mean follow-up period of 10.3 +/- 6.6 years. The longest time between the trauma and time of surgery was 43 years. Postoperative uveitis was observed in only one patient (0.5%) who had primary repair after injury. Histopathological review did not reveal typical sympathetic ophthalmia. Complications after enucleation were significantly higher than after evisceration. CONCLUSION: There was no histopathologically supported sympathetic ophthalmia in our series. Sympathetic ophthalmia is very rare even when the injured eye is retained. With this in mind, the first choice of treatment may not be enucleation. Furthermore, evisceration may be the procedure of choice if primary repair cannot be performed or for patients who have panophthalmitis.

Eye Enucleation↗

Changing patterns of intraocular inflammatory disease in Japan.

PURPOSE: We investigated the frequencies and clinical characteristics of Japanese patients with uveitis. METHODS: Records of 189 patients referred from April 1999 to March 2001 were retrospectively reviewed. RESULTS: Fifty-six patients (29.6%) had anterior uveitis, 13 (6.9%) intermediate uveitis, 59 (31.2%) posterior uveitis, 58 (30.7%) panuveitis, and three (1.6%) papillitis. The most common diagnoses were Vogt-Koyanagi-Harada (VKH) disease (10.1%), biopsy-proven or presumed sarcoidosis (9.5%), acute anterior uveitis (7.9%), tuberculosis (6.9%), and Behçet's disease (5.8%). Seventy-three patients (38.6%) were treated with local therapy alone, and 95 patients (50.3%) required systemic therapy. Ocular complications developed in 19.6% of patients, and systemic complications in 2.1%. CONCLUSIONS: These results confirm a continued high frequency of VKH disease and sarcoidosis, but suggest a decreased frequency of Behçet's disease and an increased frequency of tuberculosis. Roughly one-half of the patients required systemic treatment in addition to local therapy, and ocular and/or systemic complications developed in one-fifth of the patients.

Adolescent↗

Uveitis referral pattern in a Midwest University Eye Center.

We determined the referral pattern and visual prognosis for patients with uveitis at a tertiary university eye center in the Midwestern U.S. over a five-year period. A retrospective chart review was conducted on 853 patients seen in the Department of Ophthalmology and Visual Sciences (DOVS) at Washington University and the Barnes Retina Institute (BRI) from 1990 to 1995. Recorded data on each patient included demographic information, anatomic diagnosis, etiologic diagnosis, pattern of uveitis, and best-corrected visual acuity (VA). Most patients originated from Missouri (65.3% [557/853]) and were Caucasian (88.4% [754/853]). There was an equal gender frequency (429 men, 424 women). The most frequent anatomic diagnosis was posterior uveitis (48.4% [414/853]), followed by anterior uveitis (22.3% [190/853]), panuveitis (18.4% [155/853]), and intermediate uveitis (10.9% [94/853]). An etiologic diagnosis was made in 69.3% (591/853) of the patients, with the most common diseases being cytomegalovirus (CMV) retinopathy, infectious endophthalmitis, pars planitis, multifocal choroiditis, and toxoplasmosis. The majority of uveitides presented with sudden onset (55.0% [469/853]) and lasted less than three months (51.9% [443/853]). A wide spectrum of uveitic disease is seen in a university eye center in the Midwest, including many cases with sudden-onset relatively short (<3 months) duration. The high frequency of posterior uveitis may be related to the appearance of newly recognized uveitic entities, such as multifocal choroiditis, birdshot retinochoroidopathy, and acute retinal necrosis.

Academic Medical Centers↗

Ciliary body melanoma masquerading as chronic uveitis.

PURPOSE: To report a case of ciliary body melanoma that masqueraded as chronic uveitis. METHODS: A patient experienced persistent unilateral panuveitis and elevated intraocular pressure. Inflammation and pressure worsened after cataract extraction and lens implantation. Diagnostic vitrectomy was non-revealing. RESULTS: Repeat vitrectomy and lens implant removal revealed a ciliary body mass, suggestive of melanoma. Ultrasonography and clinical evaluation by the retinal oncologists supported this diagnosis. The uveitis persisted despite aggressive medical treatments, including methotrexate. Proton beam irradiation of the melanoma led to resolution of uveitis and restoration of normal intraocular pressure. CONCLUSION: Ciliary body melanoma can present as chronic uveitis and refractory glaucoma.

Cataract Extraction↗

Uveitis with autoimmune hepatic disorders.

Many cases of uveitis are thought to be autoimmune in nature. We report two cases of panuveitis, one of which was associated with chronic active hepatitis and the other with primary biliary cirrhosis. This suggests that the autoimmune disturbance may have been responsible for the development of the intraocular inflammation.

Administration, Oral↗

Neuro-Behçet's disease--rapidly reversible with early diagnosis and treatment.

Behçet's disease is an idiopathic multi-systemic, inflammatory disorder that may affect major organs, including the central nervous system. It is a clinical diagnosis, and a history of recurrent oral and genital ulcers should be elicited, especially in cases of unexplained uveitis with atypical neurological symptoms. We describe the rapid neurological recovery and neuroimaging changes following high-dose systemic corticosteroids in a 44-year-old man with recurrent oral ulcers, panuveitis, and acute neuro-Behçet's, presenting with meningoencephalitis. Early diagnosis with the aid of appropriate neuroimaging and immunosuppressive therapy prevented long-term neurological complications. Appropriate neuroimaging can facilitate early diagnosis and treatment, and provide prognostic information on neuro-Behçet's.

Adult↗

Causes of uveitis in a referral hospital in Ankara, Turkey.

PURPOSE: To evaluate the etiology of uveitis and its associated systemic findings in a referral hospital in Ankara, Turkey. MATERIALS AND METHODS: Clinical records of patients in our uveitis department were reviewed retrospectively and 419 eyes of 300 consecutive patients were included in the study. Cases of uveitis due to postoperative endophthalmitis, perforating injuries, and foreign bodies were excluded from the study. The data, including sex, age, clinical presentation, disease course and outcome, ocular involvement, etiology, and associated systemic findings, were entered into a computer database. Pearson chi-square statistical analysis was performed to analyze the data whenever necessary. RESULTS: The study subjects consisted of 162 males and 138 females, with a female to male ratio of 1:1.17 and an average age of 35.7 years. Anterior uveitis was the most common form of uveitis with 43.6%, followed by posterior uveitis (26.6%), panuveitis (20.6%), and intermediate uveitis (9%). Idiopathic uveitis (28.3%) was the most frequent cause of uveitis diagnosed, followed by Behçet's disease (26%) and rheumatic diseases (9%). In 215 (71.7%) out of 300 patients, a specific cause of uveitis with associated systemic findings was identified. Uveitis was attributed to an infectious etiology in only 41 patients (13.6%) of the entire study population. COMMENTS: The etiology of uveitis and its associated systemic findings may vary depending on the geographic distribution of patients and local factors. These results represent those of patients with uveitis referred to hospitals in central Anatolia.

Adolescent↗

Combined orbital floor betamethasone and depot methylprednisolone in uveitis.

PURPOSE: To evaluate the outcome of orbital floor injection of betamethasone and methylprednisolone in the management of uveitis, with particular reference to its efficacy in avoiding increase in systemic immunosuppressive drugs. METHODS: A sample of all patients attending the Uveitis Service at Moorfields Eye Hospital was carried out over a three-month period. All patients who had received one or more orbital floor injections of betamethasone (4 mg) and methylprednisolone (Depo-Medrone; 40 mg) in the management of their uveitis were identified and the case notes reviewed. RESULTS: A total of 48 patients who had received 75 orbital floor injections were identified during the sample period. Most patients had either panuveitis (n = 24) or pars planitis (n = 14). One-month post-injection visual acuity (VA) had improved by at least one Snellen line after 33/75 injections (44%) and at least two lines after 11/75 (15%), was unchanged after 25 (33%), but had deteriorated in 14 (19%). VA was not recorded after 3/75 injections. The only complications recorded were periorbital haemorrhage (n = 1) and complaints of persistent pain after an injection (n = 1). Symptoms (pain and subjective vision) improved after 36/75 injections (47%), were unchanged after 24 (32%), and were reported as worse after five (7%). Additionally, three patients reported an improvement in symptoms which lasted less than one month. Signs other than VA were improved after 28/75 (37%), were unchanged after 38 (51%), and deteriorated after only one injection. Of the 31 patients given orbital floor injections to avoid systemic therapy, seven (23%) subsequently went on to require it, and the clinical course then improved in 45%. CONCLUSIONS: Combined orbital floor injection of betamethasone and methylprednisolone can result in improved visual acuity, symptoms, and signs of inflammation in uveitis, and may therefore avoid the necessity for increased systemic medication.

Adolescent↗

Safety and efficacy of intravitreal triamcinolone acetonide for uveitic macular edema.

BACKGROUND: To evaluate the safety and efficacy of intravitreal triamcinolone acetonide (TA) for treating macular edema secondary to non-infectious uveitis. METHODS: Retrospective review of sixteen patients (20 eyes) with chronic cystoid macular edema (CME) as a consequence of controlled intermediate uveitis, posterior uveitis, or panuveitis who received at least one intravitreal injection of TA. Main outcome measures were visual acuity (VA), intraocular pressure (IOP), formation or progression of an existing cataract, and CME resolution during the follow-up period. RESULTS: At last follow-up, VA showed improvement (compared to baseline) in 11 eyes (55%), deterioration in three eyes (15%), remained completely unchanged in one eye (5%), and showed improvement initially but returned to baseline levels in five eyes (25%). At last follow-up, CME had relapsed or was still present in 10 of the eyes (50%). The remaining eyes showed complete resolution of the CME, without evidence of recurrence during the follow-up time. Mean VA at last follow-up showed statistically significant improvement (p = 0.02) in nonvitrectomized eyes (mean baseline VA: 1.14 +/- 0.58; mean final VA: 0.96 +/- 0.66) compared to the almost unaltered mean visual acuity for vitrectomized eyes (mean baseline VA: 0.76 +/- 0.41; mean final VA: 0.71 +/- 0.48)(p = 0.40, paired samples t-test). Elevation of IOP was transient in all cases and responded well to topical medications, except for one patient who required placement of an Ahmed valve. Preexisting cataract progressed in three of the 15 phakic eyes (20%). One patient developed a retinal detachment and required additional surgery to reattach it. Patients were followed for a mean of 34 weeks (median: 32 weeks; range: 19-56 weeks). CONCLUSIONS: Intravitreal TA may play a role in the treatment of uveitis-related CME. Further controlled studies are necessary to test this hypothesis.

Adult↗

Pattern of childhood-onset uveitis in a referral center in Tunisia, North Africa.

PURPOSE: To analyze the pattern of childhood uveitis in a referral center in Tunisia, North Africa. METHODS: The study included 64 patients with uveitis examined at the Department of Ophthalmology of Monastir (Tunisia) from January 1994 to July 2005. All patients had a comprehensive ocular and systemic history, including an extensive review of medical systems. Complete ophthalmic examination was performed in all cases, including best-corrected Snellen visual acuity, slit-lamp examination, applanation tonometry, and dilated fundus examination with 3-mirror lens. Standard diagnostic criteria were employed for all syndromes or entities of uveitis. RESULTS: The mean age at onset of uveitis was 12.4 years. The male-to-female ratio was 0.68. The process was unilateral in 51.6% of patients. Mean follow-up was 43.2 months. Anterior and intermediate uveitis each represented 31.25% of cases, posterior uveitis 20.3%, and panuveitis 17.2%. Noninfectious uveitis (75%) was the most frequent type of inflammation. Idiopathic uveitis was found in 50% of patients. Infectious uveitis was responsible for 25% of the cases, with toxoplasmosis (14.1%) being the most frequent cause. Twenty percent of the patients had systemic associations; juvenile idiopathic arthritis was found in 6.25%. Ocular complications occurred in 74.7% of affected eyes, of which the most common were posterior synechiae (28.4%), cataract (17.9%), cystoid macular edema (19%), and optic disc edema (32.6%). Fifty-seven affected eyes (60%) had a final visual acuity more than 20/40 and nine (9.5%) had a final visual acuity less than 20/200. CONCLUSIONS: In a hospital population in Tunisia, a specific cause of uveitis in children was found in half the patients. Idiopathic intermediate uveitis was the leading cause of uveitis in our study, followed by idiopathic anterior uveitis and toxoplasmosis. Uveitis associated with juvenile idiopathic arthritis was rare. Visual prognosis appeared to be good.

Adolescent↗

Combined low dose cyclosporine and prednisone down-regulate natural killer cell-like effector functions of CD8brightCD56+ T cells in patients with active Behçet uveitis.

PURPOSE: To investigate the changes of effector-related phenotypic markers and the natural killer (NK)-like effector functions of CD8(bright)CD56+ T cells in patients with Behçet uveitis after combined cyclosporine and prednisone (Cs/Pd) treatment. METHODS: Ten patients with active Behçet panuveitis and 10 healthy controls were prospectively recruited in this study. The effector-related surface markers (CD27, CD62L, CD11b, HLA-DR, CD94, NKG2D), chemokine receptors (CXCR1, CXCR3, CCR4, CCR5), and intracellular perforin of circulating CD8(bright)CD56+ T cells were determined by flow cytometric analysis before and after two months' treatment. NK-like cytotoxicity of ex vivo CD8(bright)CD56+ T cells against K562 was measured by standard 51Cr release assay. RESULTS: The expression levels of effector-related molecules on CD8(bright)CD56+ T cells normalized after treatment. The expression levels of CXCR1 and CCR5 were down-regulated on CD8(bright)CD56+ T cells after treatment. The amounts of preformed intracellular perforin of CD8(bright)CD56+ T cells were reduced to the normal levels. Furthermore, the NK-like cytolytic capacities of CD8(bright)CD56+ T cells were decreased after treatment. CONCLUSIONS: Our results suggest that the combined Cs/Pd treatments in active Behçet uveitis may downregulate the NK-like effector functions of CD8(bright)CD56+ T cells.

Adult↗

Patterns of uveitis in a tertiary eye care center in Iran.

PURPOSE: To identify the distribution and characteristics of new uveitis referrals to a tertiary eye care center in Tehran. METHODS: A three-year prospective study was carried out to obtain information on 544 new patients referred with uveitis. A complete ophthalmologic examination was performed in all cases; a routine set of tests and an additional battery of directed workup were conducted when indicated. RESULTS: Mean age was 32.3 years. There was no significant sex predominance. The most common forms of uveitis were anterior (38.4%) vs. other anatomical forms, chronic (62.1%) vs. acute (28.3%), nongranulomatous (85.5%) vs. granulomatous (14.5%), and noninfectious (83.5%) vs. infectious (16.5%). With regard to etiology, 45.5% were idiopathic, 19.9% were due to specific ocular disease, and 37.3% were associated with systemic disorders. Behcet's disease was the most common noninfectious disease and toxoplasmosis the most common infectious entity. The most prevalent causes were idiopathic, Fuchs' heterochromic iridocylitis, and seronegative spondyloarthropathies in anterior uveitis; toxoplasmosis, Eales disease, and toxocariasis in posterior uveitis; idiopathic, sarcoidosis, and multiple sclerosis in intermediate uveitis; and, finally, Behcet's disease, idiopathic, and Vogt-Koyanagi-Harada syndrome in panuveitis. The most frequent cause in patients under 16 years of age was pars planitis. Over 80% of the patients belonged to middle-to-upper socioeconomic classes. Uveitis significantly affected patients' lives in 63.1% of the cases. CONCLUSION: Although the current study was performed at a referral center, it may reflect to some extent the different distribution of uveitis in Iran and probably other Middle Eastern countries. Some entities such as presumed ocular histoplasmosis were not found, cytomegalovirus retinitis and birdshot chorioretinopathy were extremely rare, and HLA-B27-associated iridocyclitis was less commonly observed. In contrast, Behcet's disease, Fuchs' heterochromic iridocyclitis, Eales disease, and toxocariasis were among the more prevalent entities.

Adolescent↗

Elevated levels of interleukin 6 in the vitreous fluid of patients with pars planitis and posterior uveitis: the Massachusetts eye & ear experience and review of previous studies.

INTRODUCTION: Although the exact mechanisms that lead to uveitis are not entirely known, the role of cytokines in the pathogenesis of this disease has been shown to be important. Prior studies described the presence of an array of cytokines in the intraocular fluid of patients with uveitis. Review of these studies indicate that Interleukin-6 (IL-6) is present, and animal data suggest the important role of IL-6 in the regulation of ophthalmologic immune responses. PURPOSE: We investigated whether IL-6, TNF-alpha, IL-1 alpha, beta, IL-2 are present in the vitreous of patients with active intermediate and posterior uveitis. METHODS: Vitreous specimens were collected from 23 eyes of patients with active intermediate and posterior uveitis who underwent diagnostic or therapeutic vitrectomies. TNF-alpha, IL-1 alpha and beta, IL-2 and IL-6 were measured by enzyme-linked immunosorbent assay. Eight vitreous fluid samples from eye bank eyes were used as control. RESULTS: IL-6 was higher in the vitreous of patients with uveitis compared to control samples (p = 0.0119). No TNF-alpha, IL-2, IL1-alpha or beta was detected. The levels of IL-6 did not correlate with a specific clinical diagnosis, but patients with pars planitis and panuveitis had the highest levels (p = 0.58) CONCLUSIONS: IL-6 is elevated in the vitreous of patients with active intermediate and posterior uveitis.

Enzyme-Linked Immunosorbent Assay↗

High-grade lymphoma after azathioprine treatment for Vogt-Kaganayi-Harada syndrome.

Vogt-Kaganayi-Harada (VKH) syndrome is a rare autoimmune disease characterized by panuveitis, neuropathy and aseptic meningitis. Most patients require long-term treatment with steroids and immunosuppressants. Patients may develop concurrent autoimmune diseases, especially endocrinopathies. Secondary malignancies are rare associations. We report a Chinese man with VKH syndrome presenting with multiple cranial nerve palsy and bilateral pan-uveitis, who developed disseminated high-grade B cell lymphoma after 3-year treatment with azathioprine. This is the first report of systemic non-Hodgkin lymphoma in patients with VKH syndrome. The carcinogenic properties of azathioprine on an abnormally expanded but non-clonal lymphoid system may play a role in the pathogenesis.

Adult↗

Multiple sclerosis: comparison of the human T-cell response to S100 beta and myelin basic protein reveals parallels to rat experimental autoimmune panencephalitis.

The adoptive transfer of autoreactive S100 beta-specific T cells induces experimental autoimmune panencephalomyelitis and uveoretinitis in the Lewis rat, mimicking the distribution of lesions seen in a subset of patients with multiple sclerosis. We studied the frequency and functional properties of the human T-cell response to S100 beta in eight patients (two relapsing-remitting multiple sclerosis, one chronic-progressive multiple sclerosis, two with multiple sclerosis and uveitis, two neuromyelitis optica, one panuveitis) and in seven healthy individuals, using bovine S100 beta for T-cell stimulation. Both in patients and controls, the frequency of S100 beta-specific T-cell responses was half of that obtained for myelin basic protein (MBP), and only 10% of that obtained using purified protein derivative (PPD). The stimulation indices obtained in response to S100 beta were also less than half those obtained with either MBP or PPD. However, four long-term S100 beta-specific T-cell lines were established and studied in more detail. The four T-cell lines all exhibited a CD4+, CD8-, T-cell receptor alpha beta + surface phenotype and secreted tumour necrosis factor-alpha, interferon-gamma, interleukin-10 and interleukin-4 upon antigenic stimulation, but they were heterogenous with respect to T-cell receptor usage; two T-cell lines expressed V beta 2, one V beta 6.7 and one V beta 13. Antigen-specificity was confirmed using bovine S100 beta beta and alpha beta-isoforms, as well as a recombinant rat S100 beta preparation. The response to S100 beta was shown to the HLA-(human leukocyte antigen-) DR-restricted for two of the S100 beta-specific T-cell lines. Human S100 beta-specific T-cell lines were cytotoxic, although to a lesser extent than MBP-specific T-cell lines derived from the same donors. The phenotypic and functional properties of human S100 beta-specific T-cell lines raise the possibility that these T cells are pathogenic, as they are in the rat. The low frequency and proliferative index of S100 beta-specific, as opposed to MBP-specific T-cell responses suggests that the T-cell response to this widely expressed calcium-binding protein is under more efficient regulatory control.

Adult↗

Syphilis mimicking Reiter's syndrome in an HIV-positive patient.

A 38-year-old man with HIV infection presented with panuveitis, urethritis, and a papulosquamous eruption on his palms and soles. Careful physical and laboratory examination led to the diagnosis of syphilitic keratoderma, uveitis, and balanitis. The patient was successfully treated with penicillin and prednisone therapy. Because the initial presentation was difficult to distinguish from the symptoms of Reiter's syndrome, a high degree of clinical suspicion was required to accurately diagnose syphilis, a curable and potentially fatal disease.

Adult↗