[Vasculoproliferative retinal capillaropathy and pars planitis. Presentation of 3 cases].
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PURPOSE: To review the etiologic factors and complications of uveitis in patients younger than 16 years. PATIENTS AND METHODS: Between January 1989 and December 1999 in the Department of Ophthalmology of Hacettepe University School of Medicine, 219 patients were diagnosed or observed as having pediatric uveitis. After complete ocular and physical examinations, routine and specific laboratory and radiologic investigations were performed. Medical or surgical treatment was employed when necessary. RESULTS: Of the 219 patients, 112 were girls, with a mean age of 7.4 +/- 4.2 years, and 107 were boys, with a mean age of 8.3 +/- 3.4 years. In 24.2% of the cases, no etiologic factor could be ascertained; these cases comprised the idiopathic group. Among the remaining cases, the most common etiologies were toxoplasmosis, juvenile rheumatoid arthritis (JRA), pars planitis, Behçet's disease, and Fuchs' heterochromic iridocyclitis. Anatomically, anterior uveitis was the most common form. The mean follow-up time was 37 +/- 6.2 months. Complications for which surgical treatment was employed were identified in 71 eyes (20.9%), most of which were due to JRA, pars planitis, or Behçet's disease. CONCLUSION: Uveitis in childhood may be idiopathic or most commonly due to toxoplasmosis, JRA, and pars planitis. Due to inflammation itself or to prolonged therapy especially with corticosteroids, pediatric uveitis entities (mostly JRA, pars planitis, or Behçet's disease) may result in complications necessitating a surgical approach.
Heat shock proteins with molecular weight 70 kDa (hsp70) are highly conserved immunogenic intracellular molecules. There are two main subtypes: one is expressed constitutively (hsc70), while the other is induced under stressful conditions (ihsp70). Using an ELISA directed against recombinant human ihsp70, antibody titers were determined in patients with defined ocular inflammatory conditions (Behçet's disease, Vogt-Koyanagi-Harada (VKH), pars planitis, and sarcoidosis) as well as in a group of age-matched normal volunteers. In comparison to healthy controls (n = 14, absorbance 0.269), levels were significantly elevated in Behçet's disease (n = 18; 0.412), sarcoidosis (n = 15; 0.432), and pars planitis (n = 13; 0.346), but not in VKH (n = 10; 0.263). A correlation was also noted for treatment versus no treatment in pars planitis (p = 0.028), but not in other inflammatory conditions. There was no correlation with the level of intraocular disease activity as defined by vitreous haze and vision drop. Since pars planitis is a purely ocular condition, circulating levels of ihsp antibodies likely reflect the extent of disease involvement within the eye.
PURPOSE: Behçet's uveitis is not common in western Europe and the disease presentation is less obvious than in "endemic" countries such as Turkey and Japan. This makes the diagnosis more difficult. Early diagnosis is important, as the prognosis is better if therapy is started early. New methods such as ultrasound biomicroscopy (UBM) and indocyanine green angiography (ICGA) can improve the characterisation and diagnosis of uveitis. Our purpose was to present our experience with these new methods as well as HLA-B51 testing in the appraisal of patients with Behçet's uveitis. PATIENTS AND METHODS: Patients seen by the authors between 1997 and 2001 with Behçet's uveitis or suspected Behçet's uveitis and who underwent ICG angiography or UBM were included. Symptoms and signs, results of laboratory work-up including HLA-B51 antigen testing and the delay to diagnosis, were analysed. Fluorescein and ICG angiography and UBM testing were performed according to standard protocols used for uveitis patients and their contribution towards diagnosis and management were analysed. RESULTS: Uveitis was non granulomatous in all patients. Fluorescein angiography showed moderate to severe diffuse retinal vasculitis compatible with Behçet's uveitis in all cases. HLA-B51 testing was positive in 5 of 7 tested cases, being useful to orient the diagnosis. UBM contributed to the diagnosis in all five tested cases, being the determining element in 3 patients. It allowed redirection of the diagnosis from pars planitis to Behçet's in 2 patients with poorly transparent media because it failed to show the typical pars planitis deposits. In a case originally diagnosed as Behçet's it allowed correction of the diagnosis to pars planitis because of the presence of the typical UBM pars plana depositis. ICG angiography allowed detection of choroidal vasculitis in all five tested cases. CONCLUSIONS: In Behçet's patients who did not present with a full-blown clinical picture, as they are often seen in non-endemic areas, UBM examination and HLA-B51 testing were valuable additional diagnostic elements helping to redirect the diagnosis correctly and to reduce the diagnostic delay in these patients. The hitherto unknown choroidal vasculitis shown by ICG angiography in all five investigated patients indicates that choroidal involvement probably occurs in most newly diagnosed Behçet's patients.
BACKGROUND: Although uveitis is relatively uncommon in children, its diagnosis and management present a distinct clinical challenge for the physician. An improved knowledge of disease patterns and associated morbidity will help in the care of children with uveitis. METHODS: The authors reviewed the records of 130 patients with onset of uveitis at 16 years of age or younger. The etiology of uveitis, complications encountered, treatment administered, and visual results were analyzed. RESULTS: Uveitis associated with juvenile rheumatoid arthritis (JRA) was the largest group (41.5%) followed by idiopathic uveitis (21.5%) and pars planitis (15.3%). Twenty-six percent of the eyes had less than 20/200 visual acuity at the time of first referral. Patients with JRA had the highest rate of complications: cataract (71%), glaucoma (30%), band keratopathy (66%), and hypotony (19%). The most frequent complication of pars planitis was maculopathy (55%). Final visual acuity was less than 20/200 in 26% of eyes with JRA, 10.5% with pars planitis, and 14% with idiopathic uveitis. CONCLUSION: Uveitis beginning in childhood is a serious disease associated with sight-threatening complications. Juvenile rheumatoid arthritis-associated uveitis remains a leading cause of ocular morbidity in patients with childhood uveitis. Increased awareness by pediatricians, rheumatologists, and ophthalmologists of the seriousness of ocular complications of uveitis in childhood may lead to earlier diagnosis and more effective treatment regimens in the future.
AIMS: To determine the levels of IgG class antibodies to recombinant heat shock protein 60 kDa of Yersinia enterocolitica (rHSP60Ye), Klebsiella pneumoniae (rHSP60Kp), Escherichia coli (rHSP60Ec), Shigella flexneri (rHSP60Sf), and Streptococcus pyogenes (rHSP60Sp) in the serum of patients with HLA-B27 associated acute anterior uveitis (HLA-B27 associated AAU), idiopathic acute anterior uveitis (idiopathic AAU), pars planitis, Vogt-Koyanagi-Harada (VKH), and healthy subjects. METHODS: The genes that code for HSP60Ye, HSP60Kp, HSP60Ec, HSP60Sf, and HSP60Sp were cloned by PCR from genomic DNA. The rHSPs were purified by affinity using a Ni-NTA resin. The serum levels of IgG class antibodies to rHSP60s were determined by ELISA in patients with uveitis (n = 42) and in healthy subjects (n = 25). RESULTS: The majority of patients with uveitis had higher levels of IgG class antibodies to rHSP60Ye compared with levels of healthy subjects (p = 0.01), although these differences were only observed in the HLA-B27 associated AAU (p = 0.005) and in pars planitis patients (p = 0.001). The levels of IgG antibodies to the rHSP60Kp, rHSP60Sf, rHSP60Ec, and rHSP60Sp were similar in patients with uveitis and in healthy subjects (p>0.05). CONCLUSION: The results suggest that HSP60Ye could be involved in the aetiology of HLA-B27 associated AAU and pars planitis.
PURPOSE: Previous epidemiologic studies of uveitis have focused on predominantly Caucasian populations, and none have been based in the Southeast. We analyzed the epidemiology of uveitis among a referral population with a high percentage of African Americans in the United States. METHODS: We evaluated demographic data from 385 consecutive patients referred to the Duke Uveitis Clinic. RESULTS: Of the 385 patients, 120 (31%) were African American and 258 (67%) Caucasian; 237 (62%) were female and 148 (38%) male. The most common diagnoses among the 385 patients were idiopathic panuveitis (64 patients [17%]), idiopathic anterior uveitis (47 patients [12%]), pars planitis (46 patients [12%]), sarcoidosis (44 patients [11%]), and toxoplasmosis (39 patients [10%]). These diagnoses were also the most common among the 120 African American patients: 33 patients (28%) had idiopathic panuveitis, 30 (25%) had sarcoidosis, 10 (8%) had idiopathic anterior uveitis, 8 (7%) had toxoplasmosis, and 6 (5%) had pars planitis. Among the 258 Caucasian patients, the most common diagnoses were pars planitis (39 patients [15%]), idiopathic anterior uveitis (37 patients [14%]), toxoplasmosis (30 patients [12%]), idiopathic panuveitis (28 patients [11%]), and multifocal choroiditis and panuveitis (MCP) (17 patients [7%]). Categorizing diagnoses of all 385 patients by anatomic location, panuveitis was most frequent (148 patients [38%]), followed by anterior uveitis (97 patients [25%]), posterior uveitis (93 patients [24%]), and intermediate uveitis (47 patients [12%]). CONCLUSIONS: The higher frequency of sarcoidosis and idiopathic panuveitis than previously reported is related to our larger African American population base. The racial composition of the Southeast does not, however, account for differences such as our higher percentage of MCP; it is possible that other genetic or environmental factors play a role in this region.
Long term followup in 52 uveitis patients treated with Cyclosporine (CsA) is presented here. The patients included in this study all had intermediate or posterior uveitis of noninfectious etiology; and all were considered therapeutic failures to systemic corticosteroids and/or cytotoxic agents. Patients were considered a therapeutic success if the visual acuity in either eye improved two lines or more and/or the vitreal haze improved more than two grading levels. While five patients stopped therapy before the three month interval, 41 of 52 patients or 79% were therapeutic successes at three months after the initiation of CsA as the sole systemic immunosuppressive agent for their disease. Twenty-five of a potential 35 patients (71%) remained on CsA after one year's time, with 63% (22/35) of these individuals receiving the medication considered a therapeutic success. The patients who appeared to respond particularly well to CsA therapy were those with pars planitis, intermediate uveitis of the non-pars planitis type, VKH, and Behcet's disease. In vitro S-antigen (S-Ag) testing did not appear to have a predictive value in determining which patients would be therapeutic successes (improvement in visual acuity), however the S-Ag responder group did appear to have a statistically greater improvement in vitreal haze. Renal toxicity was by far the most common adverse effect, which we have found to be reversible with our approach to therapy. However, adverse reactions required a reduction in CsA dosage in some patients, making it not feasible to maintain therapeutically effective CsA levels. In those patients, low dose prednisone was added to these patients' CsA regimens with generally good results. We did not observe opportunistic infections nor CsA associated neoplasms in our patients. This long term study demonstrates the potential efficacy of CsA in the treatment of severe intra-ocular inflammatory disorders. However, we firmly believe that only in the context of a randomized clinical trial that a definitive evaluation as to the true effectiveness of CsA will be reached.
PURPOSE: Laser flare photometry is a new quantitative method for evaluating aqueous flare, making flare the only inflammatory parameter that can be evaluated precisely and objectively. The validity of the method already has been demonstrated in anterior segment inflammation. The aim of this study is to assess the validity and limitations of the method to quantify and monitor inflammation in uveitis with predominant involvement of the posterior segment. METHODS: Five well-defined conditions with uveitis predominant in the posterior segment were analyzed in this study: Behçet uveitis, pars planitis, posterior sarcoidosis, posterior pole toxoplasmosis, and birdshot chorioretinopathy. (1) Mean initial (pretreatment) flare was determined; (2) in the patients needing systemic steroid therapy, introduction of therapy was correlated with evoluting laser flare photometry; and (3) in patients with quiescent disease, the predictive value of a defined subclinical photometry-detected flare rise for disease recrudescence was analyzed. RESULTS: Initial pretreatment flare was 331.8 +/- 47.7 photon counts per millisecond (ph/msecond) (mean +/- standard error of the mean) for Behçet uveitis, 15.6 +/- 1.3 ph/msecond for pars planitis, 26.9 +/- 4.6 ph/msecond for posterior sarcoidosis, 7.5 +/- 1.0 ph/msecond for posterior pole toxoplasmosis, 5.8 +/- 0.7 ph/msecond for birdshot chorioretinopathy, and 4.7 +/- 0.1 ph/msecond for a group of 88 control eyes. A significant flare reduction after start of steroid therapy was seen in Behçet uveitis (78% reduction), sarcoidosis (44.8%), and pars planitis (51%), but not in toxoplasmosis or in birdshot. A small flare rise had a predictive value for disease recrudescence in 27/35 patients (predictive value, 0.77; sensitivity rate, 100%). The level of associated blood-aqueous barrier disruption for reliable follow-up of posterior uveitis was empirically determined to be 13 to 15 ph/msecond. CONCLUSION: Laser flare photometry was found to be very sensitive to monitor inflammation in uveitis of the posterior segment as long as a sufficient level of associated blood-aqueous barrier disruption (flare) was present.
The continuous curvilinear capsulorhexis has rapidly increased in popularity as the procedure of choice when using phacoemulsification for cataract extraction. Only recently, however, have complications of this technique been reported. We review the complications of a continuous curvilinear capsulorhexis and present three cases involving progressive constriction of the postoperative anterior capsular opening. One patient had a history of myotonic muscular dystrophy, another had pars planitis, and the third had high myopia. A review of ocular findings in myotonic dystrophy and pars planitis is also presented, and the possible pathophysiology of this progressive constriction is explored.
BACKGROUND/AIMS: To describe the clinical characteristics and course of 16 patients with uveitis associated with multiple sclerosis (MS). METHODS: The records of 1254 patients with uveitis were reviewed. Sixteen of these patients had MS. The history, review of systems, ocular findings, and clinical test results of each of these 16 subjects were analyzed. The mean follow-up time was 38 months. RESULTS: Most patients with MS-associated uveitis were white females between 20 and 50 years of age. The diagnosis of MS preceded the onset of uveitis in 56%, followed it in 25%, and was made concurrently in 19% of the cases. In 94%, the uveitis was bilateral. Pars planitis was the most frequent form of uveitis in our study population (81%); concomitant anterior chamber inflammation was common and was granulomatous in nature 56% of the time. Forty-one percent of the eyes with MS-associated uveitis had 20/30 or better initial visual acuity. Among these treated patients, significant loss of visual acuity was uncommon. CONCLUSIONS: MS-associated uveitis should be suspected in white female patients with bilateral uveitis, especially if pars planitis is present. These patients often retain useful vision for many years if treated.
PURPOSE: To report ultrasound biomicroscopic features in peripheral retinovitreal Toxocara uveitis. METHODS: Three patients with unilateral vitreitis suspected to be caused by peripheral toxocariasis were referred for ultrasound biomicroscopic examination of the peripheral retina, pars plana, and adjacent vitreous. RESULTS: In two of the three patients, characteristic pseudocystic transformation of the peripheral vitreous was seen. In both patients, Toxocara serology was positive and eosinophilia was present. In the third patient, no pseudocystic transformation of the peripheral vitreous was seen; however, there was the dense thickening adjacent to the pars plana usually seen by ultrasound biomicroscopy in pars planitis and corresponding to clinically visible snowbanks. The third patient had a negative Toxocara serology and later developed bilateral intermediate uveitis of the pars planitis type. In none of 48 patients with diverse inflammatory conditions of the retroiridal space that were examined by ultrasound biomicroscopy were the characteristic Toxocara-associated pseudocystic images seen. CONCLUSIONS: Pseudocystic transformation of the peripheral vitreous appears to be a rather specific and sensitive ultrasound biomicroscopic sign in patients with presumed peripheral toxocariasis that will likely aid diagnosis in difficult cases.