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.     

Ocular lesions in 200 patients infected by the human T-cell lymphotropic virus type 1 in martinique (French West Indies)

PURPOSE: To describe the ophthalmologic features observed in patients infected by the human T-cell lymphotropic virus, type 1 (HTLV-1) in Martinique (French West Indies). DESIGN: Prospective consecutive observational case series. METHODS: A complete ophthalmic examination was performed. PATIENTS: Of 200 patients infected by HTLV-1, 77 (38.5%) were seropositive and 123 (61.5%) had HTLV-1 associated myelopathy/tropical spastic paraparesis (HAM/TSP). RESULTS: Uveitis was found in 29 cases (14.5%). Symptoms were mild and the uveitis had little effect on visual function. Ten cases of uveitis were discovered through a systematic examination and had no ocular symptoms. Most of the uveitis was anterior or intermediate. The lesions responded to corticosteroid therapy, but tended to recur. Keratoconjunctivitis sicca existed in 74 patients (37%), accompanied by lymphoplasmocytoid infiltration of the secondary salivary glands rated 3 or 4 on the Chisholm scale in nearly 50% of cases. Corneal alterations were observed in 20 cases (10%), and alterations in the retinal pigment epithelium in 3 cases. CONCLUSION: The three types of ocular affections seen most frequently were uveitis, keratoconjunctivitis sicca, and interstitial keratitis. In patients with HAM/TSP, uveitis was more frequent among younger patients, patients with earlier onset of HAM/TSP, and patients with severe motor disability. Because uveitis is related to a high intrathecal production of immunoglobulin, it could represent a marker for severity of HTLV-1 infection with respect to the course of HAM/TSP. The sicca syndrome related to HTLV-1 virus differs from primary or secondary Sj?gren syndrome, because it does not reveal any of the immunologic anomalies generally seen in this disease. Interstitial keratitis was more frequent among patients with HAM/TSP who had high proviral DNA levels.     

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.     

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.     

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.     

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.     

Referral Patterns,Demographic and Clinical Features,and Visual Prognosis of Turkish Patients with Sarcoid Uveitis

The purpose of this study was to investigate the referral patterns, demographic and clinical features, and visual prognosis of patients with sarcoid uveitis seen at 2 tertiary eye care centers in Turkey. A retrospective study was made of 44 patients with uveitis associated with biopsy-confirmed or presumed sarcoidosis. Thirty-four patients (77%) were female and 10 (23%) were male. The mean age at onset of uveitis was 39.8 years. Twenty-seven patients (61%) were referred without a diagnosis of sarcoidosis and 17 (39%) patients were referred with a diagnosis of systemic sarcoidosis. The duration of uveitis prior to referral was 2–15 years in 52% of the patients in the former group. At presentation, 37 patients (84%) had bilateral and 7 patients (16%) had unilateral uveitis, and 17 patients (39%) had only anterior uveitis. The most common ocular findings were granulomatous keratic precipitates, persistent posterior synechiae, and snowball vitreous opacities. Kaplan-Meier survival analysis estimated the risk of recurrence as 30% at 6 months and 61% at 5 years. New complications developed in 18 patients. Potential visual acuity was better than 0.4 in 80% of eyes. The estimated risk of losing potential visual acuity by at least 2 lines was 7% at 6 months and 43% at 5 years. None of the eyes lost useful vision (> 0.1) under the authors' care. The referral patterns suggest underrecognition of sarcoidosis as a cause of uveitis in Turkey. The demographic and clinical features of these patients were mostly similar to those reported from other countries, suggesting that the diagnosis may not be difficult in Turkish patients but requires an increased awareness of the disease in this population.     

Referral patterns, demographic and clinical features, and visual prognosis of Turkish patients with sarcoid uveitis

The purpose of this study was to investigate the referral patterns, demographic and clinical features, and visual prognosis of patients with sarcoid uveitis seen at 2 tertiary eye care centers in Turkey. A retrospective study was made of 44 patients with uveitis associated with biopsy-confirmed or presumed sarcoidosis. Thirty-four patients (77%) were female and 10 (23%) were male. The mean age at onset of uveitis was 39.8 years. Twenty-seven patients (61%) were referred without a diagnosis of sarcoidosis and 17 (39%) patients were referred with a diagnosis of systemic sarcoidosis. The duration of uveitis prior to referral was 2-15 years in 52% of the patients in the former group. At presentation, 37 patients (84%) had bilateral and 7 patients (16%) had unilateral uveitis, and 17 patients (39%) had only anterior uveitis. The most common ocular findings were granulomatous keratic precipitates, persistent posterior synechiae, and snowball vitreous opacities. Kaplan-Meier survival analysis estimated the risk of recurrence as 30% at 6 months and 61% at 5 years. New complications developed in 18 patients. Potential visual acuity was better than 0.4 in 80% of eyes. The estimated risk of losing potential visual acuity by at least 2 lines was 7% at 6 months and 43% at 5 years. None of the eyes lost useful vision (> 0.1) under the authors' care. The referral patterns suggest underrecognition of sarcoidosis as a cause of uveitis in Turkey. The demographic and clinical features of these patients were mostly similar to those reported from other countries, suggesting that the diagnosis may not be difficult in Turkish patients but requires an increased awareness of the disease in this population.     

Visual loss in sarcoid-related uveitis

Purpose: To assess the visual acuity outcome after treatment, in patients with differing types of uveitis associated with sarcoidosis, and to determine the types of sight‐threatening complications. Methods: This was an retrospective, non‐comparative, interventional study of patients who had uveitis with a confirmed diagnosis of sarcoidosis and a minimum follow‐up duration of 6 months from presentation. The worst affected eye at presentation was selected for the study. Complications causing impaired vision were documented, changes in visual acuity after completion of treatment were measured, and the risk of ‘poor outcome’ (visual acuity 6/12 and less) was compared in the various types of uveitis, using ‘exact’ multiple logistic regression to control confounding by other prognostic factors. Results: Seventy‐five patients were studied. The spectrum of ocular inflammation in sarcoidosis is wide. Presentation may be acute and non‐granulomatous or chronic and granulomatous with posterior segment involvement being varied. Poor visual outcome was significantly more frequent in multifocal choroiditis (5/7, 71.4%), and in panuveitis without multifocal choroiditis (13/28, 46.4%), compared to only 3/24 (12.5%) patients with anterior uveitis. The excess risks remained significant after adjustment for confounding effects of other prognostic factors including age, sex and manifest systemic sarcoidosis. Causes of visual loss were cataract, glaucoma, macular oedema, vitreous haemorrhage and retinal detachment. Conclusion: Multifocal choroiditis is a less common manifestation of uveitis associated with sarcoidosis but appears to be associated with a worse visual prognosis despite immunosuppressive therapy.     

Increased number of circulating HTLV-1 infected cells in peripheral blood mononuclear cells of HTLV-1 uveitis patients: a quantitative polymerase chain reaction study.

AIMS--This study aimed to characterise the status of viral infection in patients with HTLV-1 uveitis (HU) by quantifying the circulating HTLV-1 infected cells in the peripheral blood. METHODS--Genomic DNA samples of peripheral blood mononuclear cells (PBMC) were obtained from 25 patients with HU, 14 patients with tropical spastic paraparesis/HTLV-1 associated myelopathy (TSP/HAM), and 21 asymptomatic carriers of HTLV-1. Quantitative polymerase chain reaction (PCR) of the gag region of HTLV-1 provirus DNA was performed on these DNA samples. To confirm the PCR, genomic Southern blot hybridisation was performed to identify integrated HTLV-1 provirus. This procedure detected a few percent of HTLV-1 infected cells in the PBMC. RESULTS--Most of the HU patients had a significantly increased number of circulating HTLV-1 infected cells (mean (SD) 3.84% (4.45%) of the PBMC), whereas the percentage of infected cells in most asymptomatic carriers was less than 1% (0.54% (1.11%)). Most of the TSP/HAM patients also had a relatively high percentage (11.63% (7.67%)). The differences among these three groups were highly significant by the Mann-Whitney U test. CONCLUSION--The results suggested that the increase in the number of HTLV-1 infected cells is one base for the development of inflammatory HU lesions, as it is for TSP/HAM.     

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.     

HTLV-1感染者的眼部临床表现

人类T淋巴细胞白血病病毒Ⅰ型(HTLV-1)感染比较少见,主要流行于日本、加勒比海地区、中非和南美洲。已知感染者主要眼部表现包括成人T细胞白血病(ATL)患者的眼部恶性浸润、视网膜变性、眼部神经病变,HTLV-1相关性脊髓病/热带痉挛性瘫痪(HAM/TSP)患者的干燥性角结膜炎及HTLV-1葡萄膜炎(HU)等。HTLV-1相关性眼部病变的范围正在扩大,病程中可能出现免疫调节失常引起的眼部病变或眼部肿瘤,遗传和环境因素可能在不同人群中HTLV-1患者的眼部表现起一定作用。本文就HTLV-1相关眼部表现及最新进展作一综述。     

Uveitis in Herpes zoster ophthalmicus

Background : Herpes zoster ophthalmicus (HZO) is a common condition occurring mostly in healthy people. Approximately 50% of HZO patients develop ocular complications, with iridocyclitis occurring in about 43%. This study aimed to identify the clinical features of uveitis secondary to HZO. Method : A retrospective case study was performed of consecutive patients with HZO and secondary uveitis seen in the past 10 years at the Royal Victorian Eye and Ear hospital as well as those seen in the private clinics of two ocular immunology consultants. The information collected included the time relationship between onset of rash and uveitis, duration and treatment of the uveitis, and rate and nature of ocular complications. Results : Thirty‐four patients fulfilled the enrolment criteria. The age range was from 24 to 83 years with an average age of 62.5 years. Of these, 28 patients (82%) were immunocompetent and six patients (18%) had underlying immunosuppression from various causes. Twenty‐three patients had a uniphasic episode of uveitis and 11 patients (32%) had a chronic relapsing course of uveitis. The duration of the uveitis was variable, ranging from 1 week to 3 years, with 68% of episodes resolving within 2 months. Nineteen patients (56%) developed secondary glaucoma. Five of these patients (26%) required trabeculectomies to control their intraocular pressures. Three patients (9%) had bilateral ocular involvement and five patients (15%) had a reduction in final Snellen visual acuity of more than two lines. Conclusion : In this study, most patients were immunocompetent individuals. The course of the uveitis was generally uniphasic in nature and of a relatively short duration. There was a high incidence of secondary glaucoma with 15% of all patients requiring surgical intervention. The visual loss in the five patients was not directly related to the uveitis and secondary glaucoma but to other complications associated with HZO.     

Infliximab Therapy for a Severe Case of IgG4-related Ocular Adnexal Disorder Recalcitrant to Corticosteroid Treatment

Abstract

Juvenile idiopathic arthritis (JIA) is the most common childhood rheumatic disease and the most prevalent systemic disorder in children with uveitis. The risk of developing uveitis is the greatest among patients with oligoarticular onset of disease. Clinical features of JIA-associated uveitis (JIAU) are nongranulomatous inflammation, anterior in location, insidious at onset, chronic course, and frequently asymptomatic in the absence of ocular structural complications. Uveitis in JIA can worsen over time with many sight-threatening complications, such as band keratopathy in the visual axis, posterior synechiae, cataract, secondary glaucoma, macular edema, hypotony, epiretinal membrane, and optic nerve edema. Different studies have pointed out that several factors are associated with poor prognosis, including young age at onset, male gender, short interval between diagnosis of arthritis and uveitis, severity of uveitis at onset, and antinuclear antibody (ANA) positivity. Although JIA associated-uveitis is still a serious blinding disease, we are at least able to identify the high-risk group.     

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 manifestation and visual outcomes in herpes zoster ophthalmicus: a prospective study from a tertiary hospital of Eastern India

AIM: To estimate the magnitude of different ocular manifestation in clinically established herpes zoster ophthalmicus (HZO) patients and assessment of the visual outcome after two months of initial examination. METHODS: An observational prospective study was conducted on 42 clinically diagnosed Tzanck smear positive cases HZO to observe the occurrence and frequency of different ocular manifestation and their visual outcome in 10-month period with 2mo follow up. Full ophthalmological examination using slit lamp, non-contact tonometry, applanation tonometry, direct and indirect ophthalmoscope were performed. RESULTS: Out of 42 patients of HZO, 33 had one or more type of ocular manifestation staring from lid skin involvement to conjunctivitis, keratitis, uveitis, increased intraocular pressure (IOP) and optic neuritis but no retinal manifestation. More number of HZO cases and ocular manifestation were found with advancement of ages. Young HZO patients were more associated with human immunodeficiency virus (HIV) infection and HIV infected people with HZO infection had more ocular manifestation. Male to female ratio was 2:1 among HZO cases but ocular manifestation occurred more among males. Female with advanced age were involved more. Lid involvement (73.81%), conjunctivitis (69.05%), and keratitis (59.52%) were most common ocular manifestation followed by anterior uveitis (30.95%) and episcleritis (11.90%). Ocular hypertension (42.86%) was associated with almost every ocular manifestation. Among the cases of more than 45 years of age, 9.52% patients acquired 6/6 vision compared to 7.14% patients at and below 45 years of age after 8wk of follow up. CONCLUSION: The visual outcomes are poor in HZO with advanced age group. Visual outcome of the affected eyes is poor than unaffected eyes. The loss of vision is mainly due to keratitis, anterior uveitis, posterior uveitis, and optic neuritis.     

Risk and prognostic factors of poor visual outcome in Behcet's disease with ocular involvement

Purpose The purpose of the study was to determine factors correlated with the progression of irreversible visual disturbance in Behcet's disease (BD) with ocular involvement. Methods Forty-seven BD patients with ocular inflammation, who presented with the first ocular episode, and who had been followed continuously for 5–10 years in our hospital, were studied. Charts were reviewed for gender, onset age of uveitis, complete or incomplete type BD, HLA-B51 status, final visual acuity at the last remission period, mean number of ocular attacks per year, and clinical findings of iridocyclitis with profuse hypopyon, strong vitreous opacity blocking the observation of retinal vessels, diffuse retinal vasculitis, and exudates with hemorrhage within the retinal vascular arcade. Results Patients with a visual acuity of ≤20/200 and those with >20/200 differed significantly in the mean number of ocular attacks per year and clinical findings of strong vitreous opacity and exudates within the retinal vascular arcade, but not with regard to the other factors. In addition, the frequency of ocular attacks showed a significant negative correlation with the outcome of visual acuity. Logistic regression analysis indicated a significant association of an average of more than three ocular attacks per year, strong vitreous opacity, and exudates within the retinal vascular arcade with poor visual outcome. Conclusions This study indicates that more than three ocular attacks per year, strong vitreous opacity, and exudates within the retinal vascular arcade are the risk and prognostic factors for a poor outcome of visual acuity in BD patients.     

Visual loss in uveitis of childhood

AIMS: To investigate the manifestations and severity of uveitis in children and to identify the risk and specific causes of blindness in this population. METHODS: Retrospective study of data of 123 consecutive patients examined with active uveitis and the onset of ocular disease before the age of 16 years. Numerous variables were assessed including age and sex distribution, laboratory data, the presence of systemic diseases, onset and course of ocular inflammation, clinical features and complications, therapeutic strategies and their outcomes, final visual acuity, and characteristics associated with poor visual outcome. RESULTS: Systemic disease was observed in 36/123 patients (29%), with juvenile idiopathic arthritis being the most frequent (25/123, 20%). Toxoplasma retinochoroiditis was diagnosed in 12/23 patients with posterior uveitis (52%; 10% of all with uveitis). Severe intraocular inflammation required systemic drugs in 57 (46%) patients. Ocular complications were observed in 93 patients (76%), of which the most common was cataract (43/123, 35%). Intraocular surgery was required in 35 patients (28%; in total 75 procedures). Three patients (2%) became legally blind and an additional 20/121 (17%) had one legally blind eye caused by uveitis. The most frequent causes of blindness were chorioretinal scars in the macular area and glaucoma in contrast with cystoid macular oedema (CMO) in adults. CONCLUSIONS: Uveitis in childhood is a potentially blinding disease, in the majority of patients characterised by a chronic course and a high complication rate.     

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.     

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.