ORIGINAL ARTICLE Visual Outcome of Juvenile Rheumatoid Arthritis-Associated Uveitis in Adults

Purpose: Juvenile rheumatoid arthritis (JRA) is the systemic disease most frequently associated in childhood uveitis. The disease may cause several ocular complications, visual impairment, and blindness. Recent studies revealed a more favorable ocular prognosis. Our purpose was to analyze the long-term visual outcome of JRA-associated uveitis. Methods: Ocular complications and visual outcome in adult patients with JRA-associated uveitis were evaluated. Among 18 patients included in the study, uveitis was bilateral in 12 (66.7%) and unilateral in six (33.3%), for a total of 30 eyes with ocular involvement. Results: The mean durations of JRA and its associated uveitis were 24.9 and 20.5 years, respectively. All eyes (100%) had at least one ocular complication. The most frequently observed ocular complications were cataract (83.3%), band keratopathy (60%), posterior synechia (46.7%), glaucoma (33.3%), hypotony (16.7%), and macular pathology (13.3%). Final visual acuity was impaired in 40% of the eyes, poor in 20%, and totally lost in 10%. Therefore, 70% of the eyes were either visually handicapped or totally blind. Most eyes underwent at least one surgical procedure. Inflammation was active at last examination in 63.3% of eyes. All patients were still treated topically and with systemic NSAID. Sixty-one percent of the patients were using an immunosuppressive agent. Conclusion: JRA-associated uveitis still has a severe course and blinding potential. Patients suffer from uveitis and its complications even during the adulthood period. However, because our series represents a more severe subset of the disease, the outcome may be poorer than that of some other outcome studies.     

Ocular manifestations of juvenile rheumatoid arthritis in Olmsted County,Minnesota: a population-based study

Background Juvenile rheumatoid arthritis (JRA) is the most common systemic cause of uveitis in Europe and North America. The cumulative incidence of uveitis in JRA has been reported at between 8.5% and 25% in series from referral centers in the USA. There have been no population-based studies of the cumulative incidence of uveitis in JRA in the USA.Methods We performed a population-based, retrospective cohort study of patients residing in Olmsted County, Minnesota between 1 January 1960 and 31 December 2000 who met American College of Rheumatology diagnostic criteria for JRA. The patients were identified using the Rochester Epidemiology Project (REP), a surveillance and medical records-linkage system which provides access to medical records of residents of Olmsted County. Patient histories were reviewed and information regarding rheumatic and ocular disease was extracted and analyzed. The main outcome measures were: cumulative incidence of uveitis, of complications of uveitis, of keratoconjunctivitis sicca (KCS) and of adverse visual outcome.Results Of the 88 patients identified, three patients developed uncomplicated uveitis [3.4%; 95% confidence intervals [CI] 0.7–9.6%), all with pauciarticular onset JRA. Two patients developed KCS (2.3%; 95% CI 0.3–8.0%). The visual acuity of these five patients at last follow-up (mean length of follow-up 22.6 years, range 8–36 years) was 20/20. There were no patients with visual loss attributable to JRA.Conclusions In a population-based study of JRA in the United States, uveitis occurred at a lower frequency than expected. In the limited number of cases in this cohort with JRA-associated ophthalmologic complications there was no resulting loss of visual acuity.The authors have no commercial interests.     

Incidence and outcomes of uveitis in juvenile rheumatoid arthritis, a synthesis of the literature

Background Juvenile rheumatoid arthritis (JRA) is the most common systemic cause of pediatric uveitis in Europe and North America. Uveitis is commonly perceived as a frequent sequela of JRA and JRA-associated uveitis is commonly considered to have a complicated course with frequent adverse visual outcomes. Methods We performed a systematic literature search for series of consecutive patients with JRA (as defined by the American College of Rheumatology criteria) reporting on the frequency of uveitis and/or complications of uveitis, published between January 1980 and December 2004. The main outcome measures were: the cumulative incidence of uveitis in JRA, the cumulative incidence of adverse visual outcome and that of complications in JRA-associated uveitis. Additionally, the influence of gender, presence of antinuclear antibody (ANA) and disease onset subtype to the likelihood of developing uveitis were examined. Results Analysis of pooled data from the 26 eligible series suggested a cumulative incidence of uveitis in JRA of 8.3% [95% confidence intervals (CI), 7.5–9.1%]. The cumulative incidence of uveitis varied according to geographic location, being highest in Scandinavia, then the US, then Asia and lowest in India. JRA-associated uveitis was more common in pauciarticular than polyarticular onset patients [odds ratio (OR)=3.2, 95% CI, 2.33–4.36] and in ANA-positive than ANA-negative patients (OR=3.18, 95% CI, 2.22–4.54). Female gender was only a weak risk factor for the development of uveitis in JRA patients (OR=1.69, 95% CI 1.09–2.62) and was not statistically significant after considering disease onset subtypes. In JRA-associated uveitis the cumulative incidence of cumulative incidence of adverse outcome (visual acuity <20/40 OU) was 9.2% (95% CI: 4.7–15.8) of cataracts 20.5% (95% CI: 15.5–26.3), of glaucoma 18.9% (95% CI: 14.4–24.2) and of band keratopathy 15.7% (95% CI: 10.9–21.7). Conclusion The cumulative incidence of uveitis in JRA varies according to geographic location, presence of ANA, type of JRA onset and gender. Uveitis, adverse visual outcome, and complications in JRA are less frequent than commonly accepted. Presented in part at the November 2003 American Academy of Ophthalmology meeting, Anaheim, Calif., USA (PO062). Supported by a research grant from the Department of Ophthalmology, Mayo Clinic, Rochester, MN and an unrestricted grant from Research to Prevent Blindness, Inc. New York, New York.     

Prognosis of juvenile rheumatoid arthritis-associated uveitis

PURPOSE: To evaluate the clinical characteristics and the visual prognosis of uveitis in juvenile rheumatoid arthritis (JRA). METHODS: The authors examined 63 patients with uveitis and JRA observed from January 1985 to December 2000. The following characteristics of each patient were considered: age at first visit, age at onset of uveitis and arthritis, sex, laterality and localization of uveitis, ocular complications, antinuclear antibody (ANA) and human leukocyte antigen (HLA) DR11 positivity, and follow-up. A retrospective study on mid-time visual outcome and ocular complications was performed on 42 patients with more than 12 months of follow-up. RESULTS: A total of 76.2% of the patients were female, with a mean age of 8.1 years. Chronic anterior uveitis was bilateral in 77.8% of the cases and unilateral in 22.2%. Arthritis was oligoarticular at onset in 87.3% of cases, and polyarticular in 12.7%. Mean age at arthritis onset was 4.5 years and mean age at uveitis onset was 5.4 years. ANA were positive in 92% of cases and HLA DR11 was present in 36 of the 43 patients tested (83.7%). Among the 42 patients with more than 12 months of follow-up, ocular complications occurred in 90.5% of cases and the most frequent were cataract (64.4% of eyes) and band keratopathy (59.2% of eyes). Secondary glaucoma (25% of eyes) was associated with the worst visual prognosis. A total of 64.5% of eyes maintained a visual acuity between 20/33 and 20/20 at the end of the follow-up. CONCLUSIONS: Visual prognosis of uveitis associated with JRA is improving, owing to earlier diagnosis and intensive treatment. Ocular complications occurred frequently in patients with uveitis and JRA but they did not seem to seriously affect the final visual outcome. The authors did not observe any correlation between prognosis and sex, age at the onset of uveitis or arthritis, pattern of arthritis, or positivity for ANA or HLA DR11. In a percentage of cases, uveitis may develop before arthritis or years after the onset of arthritis; therefore, continuous ophthalmologic examinations are needed in young people with JRA.     

Cataract surgery in children with chronic uveitis

OBJECTIVE: To evaluate the visual outcome of cataract surgery in children's eyes with chronic uveitis and the feasibility of intraocular lens (IOL) implantation in these cases. DESIGN: Retrospective noncomparative case series. PARTICIPANTS: Seventeen children (20 eyes) with chronic uveitis, dense cataract, and a preoperative visual acuity of 6/120 or less with follow-up of 5 years after the initial cataract surgery. METHODS: In 10 eyes of 10 children (five with juvenile rheumatoid arthritis [JRA] and five with non-JRA-associated uveitis) with uniocular or markedly unequal binocular disease, surgery was carried out through the limbus and a posterior chamber IOL was implanted. In seven children (10 eyes), three with JRA and four with non-JRA-associated disease, a pars plana approach was used, and contact lenses or glasses (for the bilateral cases) were prescribed. RESULTS: The postoperative course and immediate restored visual acuities were similar whether an IOL was implanted or not. One month after the surgery, visual acuity improved in all operated eyes. After monocular surgery, in the younger children, contact lenses were poorly tolerated and their use discontinued. These aphakic eyes remained with low vision, developing strabismus on longer follow-up. Children with JRA-associated uveitis were younger, demonstrated an active intraocular inflammation for an extended period after surgery, and tended to have secondary membranes develop, necessitating a second surgical intervention. Five years after the initial surgery, only two of nine eyes (22%) in the JRA group (one aphakic of a bilaterally affected child and one pseudophakic in a child undergoing cataract surgery in one eye) retained a visual acuity of 6/9 and 6/6, respectively. In the other seven eyes, the visual acuity was 6/60 in one pseudophakic eye and 6/240 or less in six eyes (three aphakic and three pseudophakic). In children with non-JRA-associated uveitis, 6 (four aphakic in two patients bilaterally affected and two pseudophakic) of 11 eyes (54.5%) retained a vision of 6/12 or better. CONCLUSIONS: Cataract surgery in children's eyes with uveitis may be beneficial. IOL implantation seems preferable to correction with contact lenses in young children needing surgery in one eye. In children with JRA-associated uveitis, the final visual results remain guarded because of irreversible amblyopia and a more complicated postoperative course. For these cases, a modified management approach and a better surgical technique are needed.     

Secondary glaucoma in patients with juvenile rheumatoid arthritis-associated iridocyclitis

PURPOSE: The prevalence and management of glaucoma were evaluated in patients with juvenile rheumatoid arthritis (JRA)-associated iridocyclitis. METHODS: The records of 69 patients with JRA-associated iridocyclitis were reviewed. RESULTS: Twenty-nine (42%) of these patients had secondary glaucoma or ocular hypertension. Glaucoma was controlled with topical treatment in only 7 of the 41 affected eyes (17%); systemic carbonic anhydrase inhibitor therapy resulted in control of another 8 eyes. Surgery controlled all but one of the remainder. CONCLUSION: Glaucoma is a common complication of JRA-associated iridocyclitis. It results from prolonged, inadequately treated intraocular inflammation and in some instances, from steroid use. Medical and surgical therapy for the glaucoma associated with JRA-uveitis is challenging and incompletely effective. We suspect that a more aggressive approach to the treatment of JRA-associated uveitis, earlier in the course of the disease may reduce this vision robbing contribution to the process.     

Prognostic factors in the uveitis of juvenile rheumatoid arthritis

Risk factors for significant visual loss were investigated in 51 patients with iridocyclitis associated with juvenile rheumatoid arthritis (JRA). Average follow-up was 12.7 years. Of 89 eyes with uveitis, 22% had visual loss to 20/200 or worse, 46% had cataracts, 30% had band keratopathy, and 27% had glaucoma. Severity of visual loss and complications correlated with the degree of inflammation found on initial ocular examination. Of 58 eyes that were initially normal or had signs of mild inflammation (cells, flare, keratitic precipitates), 3% had final vision of 20/200 or worse, 28% had cataracts, 5% had band keratopathy, and 17% had glaucoma. Of 31 eyes with posterior synechiae on initial examination, 58% had final vision of 20/200 or worse, 81% had cataracts, 77% had band keratopathy, and 45% had glaucoma. When arthritis clearly preceded uveitis, 6% of patients had a poor visual outcome compared to 67% of patients whose initial manifestation of JRA was uveitis. Systemic corticosteroid administration used primarily for arthritis correlated with cataract formation.     

Childhood-onset uveitis in Behçet disease:a descriptive study of 36 cases

PURPOSE: To describe the demographic and clinical features, complications, treatment, and visual results in patients with childhood-onset Beh?et uveitis. DESIGN: Observational case series. METHODS: A retrospective study was made of 36 consecutive patients with Beh?et disease seen at the Uveitis Service, Department of Ophthalmology, Istanbul Faculty of Medicine, Istanbul University, between January 1975 and January 2002. Inclusion criteria were fulfillment of the classification criteria of the International Study Group for Beh?et Disease and onset of uveitis at 16 years of age or younger. The medical records of 36 patients with childhood-onset Beh?et uveitis were reviewed. The main outcome measures were sex, age at onset of uveitis, the initial symptom of Beh?et disease, clinical ocular features, ocular complications, systemic treatment, complications of treatment, and final visual acuity. RESULTS: Twenty-five patients were male, 11 were female. Mean age at onset of uveitis was 13.6 years. The initial symptom was oral ulcer in 63.8% of the patients. The majority of patients (83.3%) had bilateral involvement. Panuveitis was the most common form (86.2%). Retinal vasculitis and retinitis were the most common ocular findings seen in 83.3% and 68.2% of the involved eyes, respectively. Cataract, maculopathy, and optic atrophy were the most common complications seen in 46.9%, 45.4%, and 39.4% of the involved eyes, respectively. Immunosuppressive therapy was administered to 75% of the patients. Response to treatment was variable. The most common complications of systemic treatment were associated with corticosteroid therapy. Final visual acuity was worse than 0.1 in 22.7% of the involved eyes. CONCLUSIONS: Childhood-onset Beh?et uveitis was more common among males. Bilateral panuveitis with retinal vasculitis and retinitis was the most common form of ocular involvement, similar to the adult patient. The treatment is challenging, as the use oral corticosteroids is associated with significant complications and the response to conventional immunosuppressive therapy is variable.     

Visual outcome in herpes simplex virus and varicella zoster virus uveitis: a clinical evaluation and comparison

PURPOSE: To compare clinical characteristics and outcomes in patients with uveitis caused by herpes simplex virus (HSV) and varicella zoster virus (VZV). DESIGN: Retrospective comparative study. PARTICIPANTS: Forty patients with HSV uveitis and 24 patients with VZV uveitis. METHODS: A retrospective study of 40 patients with HSV and 24 patients with VZV uveitis was performed. The patients were followed between May 1987 and September 1999 (median follow-up time, 46 months). The diagnosis of HSV uveitis was made clinically and serologically, and the diagnosis of VZV uveitis was made clinically. MAIN OUTCOME MEASURES: Clinical presentation of the disease, ocular complications, visual acuity, surgical and medical treatments needed. RESULTS: Both populations were comparable for gender and age at disease onset. The course of the disease tended to be remitting and recurrent in HSV patients and chronic in VZV patients (P = 0.046). The most frequent ocular complication in both groups was secondary glaucoma (54% HSV, 38% VZV). Twenty-five percent of VZV patients developed posterior pole complications (cystoid macular edema, epiretinal membrane, papillitis, retinal fibrosis, and detachment) compared with 8% of HSV patients (P = 0.069). Treatment modalities selected were generally similar in the two groups, although periocular and systemic steroids were required more frequently in HSV patients (60% versus 25%; P = 0.01). Surgical procedures were required with similar frequency in both populations. The percentage of eyes that were legally blind at end of follow-up was also comparable (HSV, 20%; VZV, 21%). The visual outcome was similar in the studied populations. CONCLUSIONS: This study represents the only direct comparison of HSV and VZV uveitis patients reported in the literature. HSV patients were more likely to be treated with periocular and systemic steroids, and VZV patients were more likely to develop posterior pole complications (a finding of borderline significance). Other parameters evaluated in this study were not statistically different in the two patient groups.     

Long-term follow-up of intermediate uveitis in children

PURPOSE: To analyze the clinical manifestations, remissions, and visual prognosis of intermediate uveitis in children, and to identify the risk factors for poor visual outcome. DESIGN: Retrospective cohort study. METHODS: Institutional study of 32 consecutive patients examined at a tertiary referral center with intermediate uveitis and the onset of ocular disease before the age of 16 years. Numerous variables were assessed, including age and gender distribution, laboratory data, the presence of systemic diseases, onset and course of ocular inflammation, clinical features and complications, therapeutic strategies and their outcomes, remission and final visual acuity, and characteristics associated with poor visual outcome. RESULTS: Bilateral involvement was observed in 94% of the patients. Remission was observed in seven out of 15 patients (47%) with completed follow-up of five years. For our 32 subjects, we found a mean time to remission of 6.4 years (SE 0.7, CI 5.1 to 7.7). Visual outcome was favorable as only three patients developed unilateral acuity of less than 0.1 after five-year follow-up, and no additional blind eyes manifested. No associated systemic diseases were established. Optic disk edema was the most frequent complication observed (71%). Cystoid macular edema (CME) was observed in 44% of the patients and was the most common cause of visual loss. CONCLUSIONS: Intermediate uveitis of childhood might exhibit a self-limiting course after several years. Visual loss was limited despite the high rate of severe ocular complications.     

Long-term progression, prognosis, and treatment of patients with recurrent ocular manifestations of Reiter's syndrome

PURPOSE: To investigate the spectrum of ocular involvement, to examine the clinical outcome, and to analyze the influence of treatment in patients with chronic ocular manifestations of Reiter's syndrome (RS) referred to a tertiary care ocular immunology service. DESIGN: Retrospective, noncomparative, interventional case series. PARTICIPANTS: Twenty-five patients with RS evaluated at the Ocular Immunology and Uveitis Service of the Massachusetts Eye and Ear Infirmary from 1981 through 2001. METHODS: Charts of patients were reviewed and data on age, gender, follow-up time, ocular symptoms, extraocular involvement, ocular complications, therapy, and visual acuities were recorded. MAIN OUTCOME MEASURES: Visual acuity, ocular complications, disease progression, clinical outcome, and systemic treatment. RESULTS: Twenty-five patients (20 male and 5 female) diagnosed with RS, with a mean age at presentation to our service of 37 years, were studied. The mean follow-up was 48.5 months. Eighty-five percent of patients tested were positive for human leukocyte antigen B27. Sixty-four percent of patients had a positive family history. All patients had oligoarthritis and enthesitis, most commonly affecting the back (56%), Achilles tendon (52%), and sacroiliac joint (24%). Eighty percent had a history of infection, most frequently urethritis (68%). Forty-four percent had a history of mucocutaneous lesions. All patients demonstrated ocular involvement at the time of diagnosis (68% with unilateral and 32% with bilateral disease), 84% had evidence of uveitis, 3% had scleritis, 2% had conjunctivitis, and 1% had pars planitis and iridocyclitis. During follow-up, the ocular complications included conjunctivitis (96%), anterior uveitis (92%), posterior uveitis (64%), keratitis (64%), cataract (56%), intermediate uveitis (40%), scleritis (28%), cystoid macular edema (28%), papillitis (16%), and glaucoma (16%). Systemic treatment for ocular inflammation was initiated in all patients. Ninety-six percent were treated with nonsteroidal anti-inflammatory agents. Eighty-eight percent were treated with corticosteroids, 64% requiring systemic prednisone. Immunosuppressive therapy was initiated in 52% of patients, with all receiving methotrexate. Seven patients required more than one immunosuppressive agent. The mean initial visual acuity was 20/25 in the right eye and 20/30 in the left eye. The mean final visual acuity was 20/25 in the right eye and 20/25 in the left eye. CONCLUSIONS: Reiter's syndrome may be associated with chronic recurrent ocular inflammation. Systemic therapy (including immunosuppressive treatment) typically is required to control the ocular inflammation and to prevent progressive visual loss.     

Spectrum of Behçet’s disease in the Indian population

The purpose of this investigation is to study the clinical profile of Behçet’s disease in the Indian population as seen in a tertiary referral uveitis clinic. A retrospective non-comparative case series was used. The records of all of the patients seen in our uveitis clinic between January 1990 and December 2006 were screened and patients with a diagnosis of Behçet’s disease were enrolled. The diagnosis was based on the Japanese diagnostic criteria and patients were classified into complete, incomplete, suspected, and possible Behçet’s disease. Information on the patient’s demographic characteristics, ocular and systemic clinical features, complications, investigation results, management protocols, and visual outcome was collected. The records of 4,214 uveitis clinic patients were screened and 53 patients (1.25%) of Behçet’s disease were identified. These included 39 males and 14 females (2.8:1), with a mean age of 27.6 years (range 10–51 years). Fifty (94.3%) of these patients were of either complete or incomplete Behçet’s disease. Ocular involvement was the most common manifestation (49 patients, 92.4%), followed by oral ulcers (88.7%), genital ulcers (61.2%), and skin involvement (28.3%). Panuveitis (57.1%) was the most common ocular presentation, followed by posterior (26.5%) and anterior uveitis (16.3%). More than 95% of the patients required oral steroids, while additional immunosuppressants were used in nearly 53% of the patients. The common complications observed included complicated cataract (57% of eyes), followed by cystoid macular edema (55.1%), occlusive vasculitis (44.8%), and optic atrophy (26.5%). Nearly 36% of the eyes maintained 6/12 or better vision, while 38% of the eyes were less than 6/60 after a mean follow-up period of 4.9 years (range 2–16 years). Behçet’s disease, although rare among Indians, commonly manifests with ocular involvement as non-granulomatous panuveitis with a high incidence of complications and unfavorable visual outcome in nearly one-third of the cases.     

Efficacy and safety of chlorambucil in intractable noninfectious uveitis: the Massachusetts Eye and Ear Infirmary experience.

PURPOSE: To report our experience with the use of chlorambucil for otherwise treatment-resistant uveitis and to assess its safety and efficacy. DESIGN: Noncomparative interventional case series. PARTICIPANTS: Twenty-eight patients with intractable noninfectious uveitis. METHODS: We reviewed the records of 28 patients (56 eyes) with chronic noninfectious uveitis who were treated with chlorambucil from 1987 to 2000. Diagnoses included Adamantiades-Beh?et's disease (ABD) (7 patients), juvenile rheumatoid arthritis (JRA)-associated uveitis (10 patients), pars planitis (2 patients), sympathetic ophthalmia (1 patient), idiopathic uveitis (6 patients), Crohn's disease (1 patient), and HLA-B27-associated uveitis (1 patient). All patients were refractory to other immunomodulatory therapy and systemic steroids. The median duration of treatment with chlorambucil was 12 months (range, 4-50 months), whereas the median daily dosage was 8 mg (range, 4-22 mg). Patients were followed for a median follow-up period of 46 months (range, 4-166 months) after chlorambucil treatment was begun and continued to be followed for relapse after cessation of therapy. MAIN OUTCOME MEASURES: Visual outcome, response to treatment, treatment-related side effects, drug dosage, previous and final treatment, discontinuation of systemic corticosteroids. RESULTS: Chlorambucil was discontinued in seven patients because of side effects: two females had temporary amenorrhea develop, two patients had unacceptable gastrointestinal intolerance, one patient had infection, and 2 patients had progressive leukopenia. Nineteen patients (68%) showed positive clinical response to the treatment, four (14%) initially responded then relapsed after discontinuation of the drug, three patients with ABD had improvement of ocular disease but worsening of systemic symptoms, and two had persistent inflammation. Visual acuity was improved in 24 eyes (43%), stable in 22 (39%), and worsened in 10 eyes (18%). Systemic prednisone was successfully discontinued in 19 of the 28 patients (68%), and 14 patients were free of inflammation at the end of follow-up without any systemic medication. CONCLUSIONS: Chlorambucil can be a safe and effective alternative for preserving vision in patients with otherwise treatment resistant uveitis.     

The development of complications in patients with chronic anterior uveitis

PURPOSE: This study aimed to identify the factors determining the ocular complications and visual outcome in patients with chronic anterior uveitis. DESIGN: Retrospective, noncomparative case study. METHODS: Ninety-one eyes of 68 patients with anterior uveitis that lasted longer than 3 months were assessed. Patients were divided into idiopathic and nonidiopathic chronic anterior uveitis and Fuchs' heterochromic uveitis. Visual acuity and complications such as posterior synechiae, cataract formation, raised intraocular pressure (IOP), and posterior segment complications (i.e., cystoid macular edema and optic nerve involvement) were evaluated. Poor visual outcome measured by life-table analysis was considered when logMAR (log of the reciprocal of Snellen visual acuity) visual acuity was 0.3 or worse. RESULTS: No statistically significant differences in the visual outcome were found between the three groups of patients with chronic anterior uveitis. Posterior synechiae formation was, as expected, significantly increased in patients with chronic anterior uveitis compared with those with Fuchs' heterochromic uveitis. There was a trend for patients with nonidiopathic chronic anterior uveitis to have an increased risk of cystoid macular edema compared with those with idiopathic disease. There were no significant differences in visual outcome between any of the groups with time. CONCLUSIONS: The visual prognosis for patients with chronic anterior uveitis is generally good. Posterior segment complications appear to be more likely to occur if there is an underlying associated disease. However, the presence of complications does not seem to affect the visual outcome in patients with chronic anterior uveitis.     

Uveitis in Behçet disease: an analysis of 880 patients

PURPOSE: We aimed to describe the demographic and clinical features, ocular manifestations, complications, visual prognosis, and treatment in a large population of Turkish patients with Beh?et uveitis. We also aimed to compare visual prognosis between male and female sex and between patients who presented before and after 1990. DESIGN: Observational case series. METHODS: A retrospective study of 880 consecutive patients (1,567 eyes) with Beh?et uveitis seen at the Uveitis Service, Department of Ophthalmology, Istanbul Faculty of Medicine, Istanbul University, from 1980 to 1998. All patients met the classification criteria of the International Study Group for Beh?et's Disease. Information on the patient's sex, age at onset of uveitis, ocular features, ocular complications, visual acuity, and systemic treatment was collected. RESULTS: Five hundred ninety-nine patients (68%) were male and 281 (32%) were female. The mean age at onset of uveitis was 28.5 years in male and 30 years in female patients. Ocular involvement was bilateral in 78.1% and unilateral in 21.9% of the patients. Panuveitis was the most common form in both sexes. Fundus lesions as well as sight-threatening complications were more common in males. At the beginning of the follow-up, potential visual acuity was 0.1 or less in 30.9% of eyes in males and 24.2% of eyes in females. Kaplan-Meier survival analysis estimated the risks of losing useful vision (>0.1) at 5 and 10 years for males and females as 21% vs 10% and 30% vs 17%, respectively. Male patients who presented in the 1990s had a significantly lower risk of losing vision compared with male patients who presented in the 1980s. CONCLUSION: Beh?et uveitis starts frequently around the end of the third decade and has a male predominance. The disease is more severe and the risk of losing useful vision is higher in males than in females. However, this risk has been significantly reduced in the 1990s.     

Risk factors for ocular complications and poor visual acuity at presentation among patients with uveitis associated with juvenile idiopathic arthritis

PURPOSE: To describe the frequencies of and risk factors for ocular complications and poor visual acuity at presentation in a cohort of patients with juvenile idiopathic arthritis (JIA)-associated uveitis. DESIGN: Cross-sectional study. METHODS: setting: Single-center, academic practice. study population: Seventy-five patients with JIA-associated uveitis were evaluated between July 1984 and August 2005. observation procedures: Data on patients diagnosed with JIA-associated uveitis were entered retrospectively into a database and analyzed. outcome measures: Visual acuity of 20/50 or worse or 20/200 or worse, and presence of ocular complications (including cataract, posterior synechiae, band keratopathy, elevated intraocular pressure, hypotony, macular edema, and epiretinal membrane) at presentation. RESULTS: At presentation, ocular complications were seen in 67% of eyes affected by JIA-associated uveitis. Presence of > or =1+ anterior chamber flare, a positive antinuclear antibody (ANA), and a shorter duration between the diagnosis of arthritis and uveitis were significantly associated with the presence of ocular complication. The frequencies of 20/50 or worse and of 20/200 or worse visual acuities at presentation in affected eyes were 36% and 24%, respectively. The presence of > or =1+ anterior chamber flare and a history of intraocular surgery before presentation were significantly associated with 20/50 or worse and 20/200 or worse vision. Presence of posterior synechiae also was associated with 20/200 or worse vision at presentation. The main causes of poor vision at presentation for affected eyes and better-seeing eyes were cataract, band keratopathy within the visual axis, and glaucoma. CONCLUSIONS: Ocular complications and poor vision at presentation were common in our patients with JIA-related uveitis.     

The effect of treatment and its related side effects in patients with severe ocular cicatricial pemphigoid.

PURPOSE: To determine the clinical outcome of patients with ocular-cicatricial pemphigoid (OCP) and the influence of systemic treatment on clinical progression. DESIGN: Noncomparative interventional case series. PARTICIPANTS: Sixty-one patients with biopsy-proven OCP. METHODS: Patients with documented disease progression treated with chemotherapy and/or corticosteroids were followed between 1985 and 2000. The parameters evaluated were ocular stage at presentation, visual acuity, ocular complications, disease progression, control of ocular inflammation, and presence of extraocular involvement. Systemic treatment and related side effects were analyzed. MAIN OUTCOME MEASURES: Visual acuity, ocular complications, extraocular involvement, disease progression, clinical outcome, systemic treatment, and related side effects. RESULTS: Sixty-one patients (32 female; 29 male) with a mean age of 67 years were studied. Extraocular involvement was present in 50% of patients. Sixty percent of eyes were initially seen with stage III (advanced cicatrizing) disease at first evaluation. Seven percent of involved eyes at first visit and 21% at the end of follow-up were legally blind. The most common ocular complications encountered were dry eye, corneal abnormalities, and glaucoma. Dapsone was the most commonly used drug (51 patients), followed by methotrexate (24 patients), azathioprine (23 patients), and cyclophosphamide (15 patients); prednisone, always given as adjunctive treatment, was used in 17 patients. Control of ocular inflammation (total or partial) was achieved in 90% of patients, but 46% of them needed continuation of systemic treatment to avoid disease recurrences, and 10% progressed despite different drugs used. Two agents were required in 32% of cases to control disease activity. The most common treatment-related side effects were hematologic complications (n = 34) followed by gastrointestinal (n = 17), cardiovascular (n = 15), and urinary complications (n = 11). Dapsone was responsible for the greatest number of side effects (n = 43); methotrexate caused the least trouble (n = 6). Corticosteroid-related complications (n = 34) were mostly cardiovascular and endocrinologic. CONCLUSIONS: Ocular-cicatricial pemphigoid is an autoimmune disease that, untreated, progresses to conjunctival scarring and blindness; systemic immunosuppression is required to control it. Long-term systemic treatment and more than one drug are frequently necessary to avoid recurrences, exposing elderly patients to a higher risk of drug toxicity. The most frequently encountered treatment-related side effects were anemia, leukopenia, liver toxicity, and hypertension.     

Koch’s postulates and the polymerase chain reaction

To investigate the clinical manifestations of human T-lymphotropic virus type-1 uveitis (HU), 112 HU patients who were followed up periodically for more than one year were retrospectively analyzed with respect to their ophthalmological and systemic complications. The gender ratio (female/male ratio) of the HU patients was 2.0 and the initial complications were foggy vision in 34.5%, ocular floaters in 33.3%, and blurred vision in 15.5%. As for the ocular symptoms, the majority (78.6%) of patients were classified as intermediate uveitis with vitreous inflammation. Recurrence of uveitis episodes was seen in one half of the patients (51.8%); 12 patients had more than six uveitis episodes. The interval of uveitis episodes varied from two weeks to 10 years. Nearly one half of the patients (43.8%) had ocular complications: e.g., cataract in 22 patients, persistent vitreous opacities in 17 patients, and glaucoma in 16 patients. Although the visual prognosis was essentially good, 11 patients had poor visual prognosis (&;lt;0.1). The causes of poor vision in these patients were cataract, cystoid macular edema, epiretinal membrane, and optic nerve atrophy. Of the 112 HU patients, two developed HTLV-I-associated myelopathy (TSP/HAM) after the onset of HU, while none developed adult T-cell leukemia. Sixteen HU patients had a previous history of Graves' disease and a past history of methimazole therapy, while Graves' disease was found in another HU patient only after HU onset and methimazole was not administered before the onset of HU. The present data of long-term follow-up indicate that (1) HU causes various ocular complications and its visual prognosis can be poor, (2) TSP/HAM can be induced even after the onset of HU, and (3) methimazole is not a risk factor of HU after Graves' disease.     

Clinical features of human T-lymphotropic virus type 1 uveitis: a long-term follow-up

To investigate the clinical manifestations of human T-lymphotropic virus type-1 uveitis (HU), 112 HU patients who were followed up periodically for more than one year were retrospectively analyzed with respect to their ophthalmological and systemic complications. The gender ratio (female/male ratio) of the HU patients was 2.0 and the initial complications were foggy vision in 34.5%, ocular floaters in 33.3%, and blurred vision in 15.5%. As for the ocular symptoms, the majority (78.6%) of patients were classified as intermediate uveitis with vitreous inflammation. Recurrence of uveitis episodes was seen in one half of the patients (51.8%); 12 patients had more than six uveitis episodes. The interval of uveitis episodes varied from two weeks to 10 years. Nearly one half of the patients (43.8%) had ocular complications: e.g., cataract in 22 patients, persistent vitreous opacities in 17 patients, and glaucoma in 16 patients. Although the visual prognosis was essentially good, 11 patients had poor visual prognosis (<0.1). The causes of poor vision in these patients were cataract, cystoid macular edema, epiretinal membrane, and optic nerve atrophy. Of the 112 HU patients, two developed HTLV-I-associated myelopathy (TSP/HAM) after the onset of HU, while none developed adult T-cell leukemia. Sixteen HU patients had a previous history of Graves' disease and a past history of methimazole therapy, while Graves' disease was found in another HU patient only after HU onset and methimazole was not administered before the onset of HU. The present data of long-term follow-up indicate that (1) HU causes various ocular complications and its visual prognosis can be poor, (2) TSP/HAM can be induced even after the onset of HU, and (3) methimazole is not a risk factor of HU after Graves' disease.