Bilateral Intermediate Uveitis Research Articles

Uveitis and multiple sclerosis : Clinical aspects, diagnostics, management and treatment

Approximately 0.5-1% of patients with multiple sclerosis (MS) have co-existing uveitis. Both intraocular inflammation and MS mainly affect women in younger adulthood. The MS in patients is most frequently associated with an often bilateral intermediate uveitis with typical concomitant retinal vasculitis. Both diseases share similar characteristics with chronic inflammatory diseases with arelapsing course and an immune-mediated pathogenesis; however, it is still unclear whether the co-occurrence of uveitis and MS in the same patient represents acoincidence of two separate disease entities or whether uveitis is arare clinical manifestation of MS. In the differential diagnostics of intermediate uveitis, clinical symptoms and signs of MS should be considered. As both diseases are considered to be immune-mediated, immunotherapy is the main treatment option. In recent years the range of medications has expanded and includes several disease modifying drugs (biologics). When selecting the active substance it must be taken into account that tumor necrosis factor (TNF) alpha blockers are contraindicated in patients with MS.

Intermediate uveitis in common variable immunodeficiency (CVID) associated with a heterozygous variant in the TNFRSF13B gene.

To report on a rare case of intermediate uveitis occurring in a patient with common variable immunodeficiency (CVID) and a heterozygous TNFRSF13B variant. Observational case report. A 23-year-old male presented with a 3-month history of increasing floaters and blurred vision to both eyes. He had been treated with topical and intravitreal corticosteroids by his local ophthalmologist nine months before. Ocular examination demonstrated bilateral intermediate uveitis with retinal vasculitis. He had been treated with intravenous immunoglobulins during childhood, due to primary humoral immunodeficiency. Systemic work-up for other causes of intermediate uveitis was unremarkable, notably no features of systemic sarcoid-like disease were detected. Initial treatment with mycophenolate mofetil showed insufficient response, and upon switching to adalimumab, clinical remission was achieved. Immunocytometry and genetic work-up revealed a smB+CD21norm subtype of CVID and a heterozygous TNFRSF13B variant. This report of CVID-associated intermediate uveitis in a patient with a heterozygous TNFRSF13B variant highlights the potential involvement of the eye within CVID-associated autoimmunity and the role for anti-TNF blockade in this challenging group of patients.

Prevalence and characteristics of HTLV-associated uveitis in patients from Bahia, an endemic area for HTLV − 1 in Brazil

BackgroundHTLV-1-associated uveitis (HAU) is an inflammatory reaction of the choroid, retina, optic nerve and vitreous that can lead to vision impairment. The worldwide prevalence of HAU varies widely.ObjectiveTo determine the prevalence of HAU in patients from Salvador, Bahia-Brazil, and describe uveitis type and associated symptoms.MethodsCross-sectional analytical study to determine the prevalence of uveitis in HTLV-1-infected patients recruited in Bahia, Brazil, a region considered endemic for HTLV-1. Patients were enrolled at a local reference center for HTLV (infected) and at an outpatient ophthalmology clinic (noninfected group). All patients were examined by the same ophthalmologist following a single protocol. Prevalence ratios (PR) were calculated.ResultsA total of 168 consecutively examined HTLV-1-infected patients and 410 noninfected patients (randomly selected) were included. Females predominated (82.1%) in the HTLV-1-infected group (versus 64.4% in the uninfected group) (p < 0.001). The mean age of infected and uninfected patients was 53.2 and 62.8 years, respectively (p < 0.001). The prevalence of uveitis in HTLV-1+ and HTLV-1− patients was 7.14% and 0.73%, respectively (PR = 9.76; 95CI%:2.79–34.15; p < 0.01). Bilateral intermediate uveitis, associated with symptoms including visual disturbances and floaters, was most commonly identified in the HTLV-1-infected patients, whereas unilateral anterior uveitis, in association with symptoms such as blurring and ocular pain, was more common in the uninfected group.ConclusionThe prevalence of uveitis in patients with HTLV-1 was markedly higher than in uninfected subjects. HAU patients were mostly asymptomatic and exhibited bilateral presentation, with uveitis more frequently localized in the intermediate chamber.

Bilateral intermediate uveitis following treatment with paclitaxel in a patient with invasive ductal carcinoma of the breast

BackgroundTo report a case of bilateral intermediate uveitis without cystoid macular edema secondary to paclitaxel therapy, and its successful management with oral corticosteroids.Case presentationA 66-year-old female developed bilateral intermediate uveitis with reduced best corrected visual acuity to 20/40 right and 20/200 left, following 12 cycles of paclitaxel therapy for breast carcinoma. Optical coherence tomography demonstrated no cystoid macular edema in either eye, and fundus fluorescein angiography showed localized retinal vascular leakage. Resolution of uveitis and improvement of visual acuity followed treatment with oral prednisolone for two months. Fourteen months after presentation, right and left visual acuities had returned to 20/32 and 20/40, respectively, and there was no recurrence of the uveitis.ConclusionsThis is the first reported case of bilateral intermediate uveitis in a patient treated with paclitaxel. Drug-induced uveitis should be considered in patients with visual symptoms in the setting of taxane chemotherapy, and oral corticosteroids are a safe and effective treatment.

Tubulointerstitial Nephritis and Uveitis Syndrome (TINU): A Case Series in a Tertiary Care Uveitis Setting.

Background: Tubulointerstitial nephritis and uveitis syndrome (TINU) is a rare disorder typically characterized by sudden-onset non-granulomatous anterior uveitis associated with tubulointerstitial nephritis (TIN). However, the prevalence and clinical features of TINU are still a matter of debate. To add information about TINU, we describe here the clinical features of a series of patients affected by TINU in a retrospective study. Methods: A total of 9358 clinical records of both adult and pediatric patients up to 21 years of age, referred to the Uveitis Center of the Sapienza University of Rome, were examined. The medical records covered a period from 1990 to 2020. Various demographic and clinical features were analyzed in patients who met the criteria for TINU. Results: Twenty-one patients with TINU were identified. TINU was classified as definite, possible, or probable by the currently recognized international criteria. The median age at diagnosis was 14 years (interquartile range 12–35). Females were predominant (15/21, 71.4%). In most cases (14/21, 66.6%), patients developed ocular disease concurrently with renal disease. The most frequent type of ocular involvement was bilateral anterior uveitis (9/21, 42.8%). In two cases, patients presented with bilateral intermediate uveitis; in three cases, they presented with bilateral or unilateral alternating posterior uveitis; and in four cases, they presented with bilateral panuveitis. In one case, the uveitis was anterior in the right eye (OD) and posterior in the left eye (OS), and two cases presented with bilateral asynchronous or unilateral alternating anterior uveitis. All patients received treatment with systemic corticosteroids and topical ocular therapy. At the end of the follow-up, a significant improvement in ocular signs and symptoms with a return to normal visual acuity was generally observed. In all patients, acute kidney injury (AKI) reverted completely and none progressed to chronic kidney disease (CKD). Conclusions: Patients with TINU may often present with atypical uveitis. We suggest that patients with sudden-onset uveitis, even if not bilateral anterior, should be referred to a nephologist for an assessment of the possible presence of renal disease.

Blau syndrome: a case report from Palestine

BackgroundThis case study documents the first familial case of Blau syndrome (BS) in Palestine characterized with mutation in CARD15/NOD2.Case presentationEighteen years old female was initially misdiagnosed with Juvenile idiopathic arthritis (JIA). The patient had been on steroids and methotrexate treatment for the last 16 years, but did not respond well to treatment. Initial examination at Saint John of Jerusalem Eye Hospital Group clinic showed bilateral intermediate uveitis with camptodactyly. The patient’s sister (aged 19 years) had bilateral intermediate uveitis and camptodactyly. Both eyes of their father had signs of old posterior uveitis. Father’s left eye showed 360 degrees posterior synechia, mature cataract with old Keratic precipitates (KPs). He also had camptodactyly. The patient was referred to pediatric rheumatologist to rule out sarcoidosis.Lung CT scan showed bronchiectasis, genetic consultation followed. Complete eye examination, full history, refraction, and Optical coherence tomography (oct) were done. Systemic and topical steroid therapy could not control the ocular inflammation. The family then was referred to a geneticist. Genetic analyses showed that the proband and all three family members had an R334q mutation in the CARD15/Nod2 gene.ConclusionsBS should be considered in the differential diagnosis of childhood uveitis, especially in low and middle income countries where it is misdiagnosed in many cases, which delay appropriate diagnosis and thus control. Genetic analysis of the CARD15/Nod2 gene is helpful in the diagnosis. Steroids alone are not enough to control the disease, other immunosuppressants and biologics are needed.

Classification Criteria for Multiple Sclerosis-Associated Intermediate Uveitis

The purpose of this study was to determine classification criteria for multiple sclerosis-associated intermediate uveitis. Machine learning of cases with multiple sclerosis-associated intermediate uveitis and 4 other intermediate uveitides. Cases of intermediate uveitides were collected in an informatics-designed preliminary database, and a final database was constructed of cases achieving supermajority agreement on the diagnosis, using formal consensus techniques. Cases were split into a training set and a validation set. Machine learning using multinomial logistic regression was used in the training set to determine a parsimonious set of criteria that minimized the misclassification rate among the intermediate uveitides. The resulting criteria were evaluated in the validation set. A total of 589 cases of intermediate uveitides, including 112 cases of multiple sclerosis-associated intermediate uveitis, were evaluated by machine learning. The overall accuracy for intermediate uveitides was 99.8% in the training set and 99.3% in the validation set (95% confidence interval: 96.1-99.9). Key criteria for multiple sclerosis-associated intermediate uveitis included unilateral or bilateral intermediate uveitis and multiple sclerosis diagnosed by the McDonald criteria. Key exclusions included syphilis and sarcoidosis. The misclassification rates for multiple sclerosis-associated intermediate uveitis were 0 % in the training set and 0% in the validation set. The criteria for multiple sclerosis-associated intermediate uveitis had a low misclassification rate and appeared to perform sufficiently well enough for use in clinical and translational research.

Epstein-Barr virus uveitis after intravitreal triamcinolone injection

Purpose: To report a case of Epstein-Barr virus (EBV) uveitis after intravitreal triamcinolone injection.&#x0D; Methods: Observational case report.&#x0D; Results: A 66-year-old male presented with bilateral intermediate uveitis, left macular branch retinal vein occlusion, and left macular edema 3 months following acute infectious mononucleosis. He received systemic prednisolone, methotrexate, and intravitreal bevacizumab with partial response. Intravitreal triamcinolone was given for recurrent macular edema, which led to the development of severe panuveitis with positive EBV PCR in aqueous humour. This was successfully treated with high-dose systemic valaciclovir and topical prednisolone.&#x0D; Conclusion: Non-infectious uveitis may become infectious following intravitreal steroid administration triggering intraocular viral replication. Intraocular fluid should be tested in cases which are suspicious for infection and EBV should be considered a differential diagnosis, particularly if PCR is negative for more common viral etiologies.

Classification Criteria For Pars Planitis

To determine classification criteria for pars planitis. Machine learning of cases with pars planitis and 4 other intermediate uveitides. Cases of intermediate uveitides were collected in an informatics-designed preliminary database, and a final database was constructed of cases achieving supermajority agreement on the diagnosis, using formal consensus techniques. Cases were split into a training set and a validation set. Machine learning using multinomial logistic regression was used on the training set to determine a parsimonious set of criteria that minimized the misclassification rate among the intermediate uveitides. The resulting criteria were evaluated on the validation set. Five hundred eighty-nine cases of intermediate uveitides, including 226 cases of pars planitis, were evaluated by machine learning. The overall accuracy for intermediate uveitides was 99.8% in the training set and 99.3% in the validation set (95% confidence interval 96.1, 99.9). Key criteria for pars planitis included unilateral or bilateral intermediate uveitis with either 1) snowballs in the vitreous or 2) snowbanks on the pars plana. Key exclusions included: 1) multiple sclerosis, 2) sarcoidosis, and 3) syphilis. The misclassification rates for pars planitis were 0% in the training set and 1.7% in the validation set, respectively. The criteria for pars planitis had a low misclassification rate and appeared to perform sufficiently well for use in clinical and translational research.

Classification Criteria for Intermediate Uveitis, Non–Pars Planitis Type

To determine classification criteria for intermediate uveitis, non-pars planitis type (IU-NPP, also known as undifferentiated intermediate uveitis). Machine learning of cases with IU-NPP and 4 other intermediate uveitides. Cases of intermediate uveitides were collected in an informatics-designed preliminary database, and a final database was constructed of cases achieving supermajority agreement on the diagnosis, using formal consensus techniques. Cases were split into a training set and a validation set. Machine learning using multinomial logistic regression was used on the training set to determine a parsimonious set of criteria that minimized the misclassification rate among the intermediate uveitides. The resulting criteria were evaluated on the validation set. Five hundred eighty-nine of cases of intermediate uveitides, including 114 cases of IU-NPP, were evaluated by machine learning. The overall accuracy for intermediate uveitides was 99.8% in the training set and 99.3% in the validation set (95% confidence interval 96.1, 99.9). Key criteria for IU-NPP included unilateral or bilateral intermediate uveitis with neither snowballs in the vitreous humor nor snowbanks on the pars plana. Other key exclusions included multiple sclerosis, sarcoidosis, and syphilis. The misclassification rates for IU-NPP were 0% in the training set and 0% in the validation set. The criteria for IU-NPP had a low misclassification rate and seemed to perform well enough for use in clinical and translational research.

Fever, uveitis, and myocarditis as the initial presentation of Behcet's Disease: A case report and review of the literature

Behcet's disease (BD) is an inflammatory disease characterized by recurrent aphthous ulcers, genital ulcers, and eye involvement. The involvement of the vascular system, heart, lungs, gastrointestinal tract, and the brain is less common, and BD myocarditis is very rare, with only a few cases reported in the literature. The prevalence of BD is variable globally, with Turkey having the highest prevalence, with 370 cases/100,000 population. Out of the 17 criteria proposed for the diagnosis of BD, the most commonly used criteria are the International Study Group (ISG) on BD criteria and the “International Criteria of BD” (ICBD) criteria. We present a case of a 40-year-old female who presented with fever of unknown origin, oral ulcers, odynophagia, and a rash over the face, extremities, and trunk. She developed acute-onset bilateral painful red eyes in the hospital with marked diminution of vision and persistent tachycardia. On evaluation, she was diagnosed to have bilateral intermediate uveitis based on the clinical finding of conjunctival congestion, and 1+ cells in the anterior chamber of both eyes, dispersion of iris pigments on the anterior surface of lens bilaterally, and Grade 1 vitreous haze on slit-lamp examination. The patient had persistent tachycardia for which an electrocardiogram was done, which revealed nonspecific ST-changes. Echocardiography was done, which showed a left ventricular ejection fraction of 40%, with global hypokinesia, suggestive of myocarditis. Our patient fulfilled the ICBD criteria, and she tested positive for human leukocyte antigen B51. She also had persistent transaminitis, and on evaluation, she was found to have occult hepatitis B infection with possible reactivation. The final diagnosis of BD with myocarditis and occult hepatitis B infection was established. She was managed with oral glucocorticoids, colchicine, azathioprine, and entecavir with a favorable outcome. BD causing myocarditis is an infrequent presentation, and we could find only 15 cases reported in the literature since 1947 when Dr. Hulusi Behcet first described the disease.

Insights into multiple sclerosis-associated uveitis: a scoping review.

This paper is a scoping review of research on multiple sclerosis (MS)-associated uveitis to determine its epidemiology, pathophysiology, clinical features and treatment. A comprehensive search of the medical databases MEDLINE (PubMed), EMBASE, Web of Science and Cochrane was carried out on 25 November 2019, to identify papers published between 1980 and 2019 that focus on patients with MS-associated uveitis. Based on large cohort studies (n≥1000), the prevalence of uveitis in patients with MS is estimated to be 0.53-1.34% (mean=0.83%), and MS is diagnosed in 0.52-3.20% (mean=1.30%) of patients with uveitis. The condition is most frequent among middle-aged women. Patients usually complain of floaters and/or blurred vision, with bilateral intermediate uveitis (with retinal vasculitis) as the most frequent ophthalmological finding. Both MS and intermediate uveitis are associated with HLA-DRB1*15:01 and IL-2RA gene polymorphism rs2104286 A>G, suggesting a common genetic background. T cells, and possibly B cells, play an important role in both autoimmune disorders. Multiple sclerosis (MS)-related uveitis is classically treated as non-infectious uveitis, with corticosteroids as the first treatment step. Other treatments include immunosuppressants, cryotherapy, laser photocoagulation and vitrectomy. These treatment options have a limited, if any, effect on the course of MS and can be complicated by side-effects. As treatment strategies for MS have increased in the last decade, it would be interesting to evaluate the efficacy of these new treatments during the course of uveitis. Moreover, the correlation between retinal periphlebitis and MS could be established more accurately with the recently developed techniques of wide-field fluorescein angiography in a large cohort of MS patients. MS-associated uveitis is a rare, highly discussed pathology about which much is still unknown. Large epidemiological studies and extrapolation of new MS treatments to this condition are warranted.

The Clinical Characteristics of Pediatric Non-Infectious Uveitis in Two Tertiary Referral Centers in Turkey

ABSTRACT Purpose: To report the manifestations, patterns of disease, treatment strategies and outcomes in pediatric patients with noninfectious uveitis. Methods: Demographic information of 76 cases was recorded. Symptoms, anatomic location, laterality, visual acuity (VA), intraocular pressure, associated systemic diseases, therapeutic strategies, side effects, complications were reviewed. Results: Thirty-one patients were diagnosed as uveitis on routine surveillance because of underlying systemic disease. The most common anatomic location was intermediate uveitis (34.2%). Juvenile idiopathic arthritis (JIA) was the most common underlying systemic disease (25%). Glaucoma was the most common complication (7.7%). The patients with refractory uveitis received adalimumab (26.5%), infliximab (4.6%) and tocilizumab (3.1%). The mean first-year VA was between 20/32 and 20/20 in 116/140 eyes. Conclusion: Most pediatric noninfectious uveitis cases have bilateral intermediate uveitis. JIA was the most common systemic association. The first-year VA was good in most eyes which may be due to early use of corticosteroid-sparing agents.

Etiologic Spectrum and Follow-Up Results of Noninfectious Uveitis in Children: A Single Referral Center Experience.

This study aims to investigate the etiologic spectrum, demographic features, and long-term follow-up results of children with noninfectious uveitis (NIU). Files of patients with NIU were reviewed between May 2010 and September 2017. The cohort consisted of 54 juvenile uveitis patients (26 males, 28 females; mean age 7.7 years; interquartile range [IQR] 9.2 years) with 93 affected eyes. Location of uveitis, laterality, age at onset of uveitis, complications of uveitis, duration of follow-up, associated systemic diseases, pertinent laboratory tests, medications used, and status of uveitis at the time of enrollment were recorded from the files. All patients had final systemic and ocular examination at the last month of enrollment. Twenty-seven patients (50.0%) had juvenile idiopathic arthritis (JIA), 17 (31.4%) had idiopathic uveitis, six (11.1%) had Behçet disease (BD), and four (7.5%) had tubulointerstitial nephritis and uveitis (TINU) syndrome. Median duration of follow-up for uveitis was 16 (IQR: 15) months. Anterior uveitis was seen in 81.4% of the patients (65.9% had bilateral and 34.1% had unilateral anterior uveitis). Bilateral intermediate uveitis was observed in 11.2% and bilateral panuveitis in 7.4% of the patients. At the time of enrollment, 45 uveitis patients (83.3%) were under remission. Complications of uveitis were observed in 18.5% of the patients. Patients with JIA and BD should be regularly checked for uveitis. It is challenging to find an etiology in uveitis patients referred from ophthalmologists if initial questioning and examination do not reveal an overt rheumatologic disease. However, a simple urine test may help in establishing the diagnosis of TINU syndrome.

INTERMEDIATE UVEITIS AS AN INITIAL PRESENTATION OF HLA B27 ASSOCIATED SPONDYLOARTHROPATHY IN AN ADOLESCENT BOY: A RARE CASE REPORT AND REVIEW OF LITERATURE

Juvenile ankylosing spondylitis is less prevalent in children, and usually, children are presented to the hospital with chronic inflammatory back pain, enthesitis, and often hip and shoulder joint involvement. A significant proportion of these children develop ocular complications such as anterior and intermediate uveitis. Most of these children have HLA B27 positivity. On the other hand, in cases with anterior and intermediate uveitis, a considerable proportion has positivity for HLA B27 antigen. Few of these patients, later on, develop other clinical features suggestive of ankylosing spondylitis. However, there are only a few anecdotal case reports of similar HLA B27 associated intermediate uveitis, who later on developed juvenile ankylosing spondylitis. The current case is a 12-year old boy, who initially had bilateral intermediate uveitis with HLA B27 positivity without any other systemic or musculoskeletal features. However, on follow-up he developed enthesis and sacroiliitis suggestive of ankylosing spondylitis. Both rheumatological and visual complains had an excellent clinical response to corticosteroids in this child. The current case report demonstrates the importance of evaluation for HLA B27 positivity in all children with isolated intermediate uveitis, even if systemic and musculoskeletal examinations are normal.

Bilateral intermediate uveitis with appearance of frosted branch angiitis and association with Mycoplasma pneumoniae infection: case report and review of the literature

A 12-year-old girl presented with acute bilateral visual loss following a mild upper respiratory tract infection. Clinical examination revealed bilateral severe peripheral retinal vasculitis with an appearance of frosted branch angiitis and cystoid macular edema. All tests for etiological diagnosis were negative, apart from positive IgM and IgG antibodies for Mycoplasma pneumoniae. She was treated with broad-spectrum antibiotics and intravenous corticosteroids. She responded to treatment, with dramatic improvement of her visual acuity, remission of angiitis, and a residual macular star formation. This is the first reported case of bilateral intermediate uveitis possibly associated with Mycoplasma pneumoniae infection.

Bilateral intermediate uveitis asociated with retinosis pigmentosa

AbstractPurpose Retinitis pigmentosa (RP) is a group of inherited dystrophies with great clinical genetic and evolutionary heterogeneity. We report a patient with retinitis pigmentosa who presented a bilateral intermediate uveitis with cystoid macular edema associated and no other systemic disease.Methods A 16 year old man with a history of Retinosis pigmentosa since birth presented blurred vision associated with a bilateral intermediate uveitis. The visual acuity was 0,4, Tyndall ++, and intense vitritis with snowballs in both eyes. All systemic examinations were normal (analytics, autoimmunity and serology tests).Results Patient was treated with orals corticosteroids maintained in decreasing doses. Response to treatment was favorable, but then macular edema appeared. Due to the inability to control uveitis symptoms with steroids, azathioprine was associated and response has been favorable with resolution of macular edema and decreasing inflammation.Conclusion RP includes a large group of degenerative and hereditary diseases that may be associated with a number of ocular complications. Intermediate uveitis is a rare complication described but can cause decreased vision and requiring monitoring and appropriate treatment for their control.

The present role of corticosteroids in uveitis

AbstractCorticosteroids are the most widely used anti‐inflammatory and immunosuppressant drugs in ophthalmology in general, and remain the mainstay of therapy for patients with uveitis.An infectious etiology for intraocular inflammation should be adequately excluded or appropriately covered with anti‐infectious therapy before administration of corticosteroid therapy. Topical corticosteroids alone are usually effective in the management of anterior uveitis and have little activity against intermediate or posterior uveitis. Ocular adverse effects of topical steroid therapy mainly include ocular hypertension and cataract. The use of periocular steroid injections (subconjunctival, anterior or posterior subtenon, orbital floor) are important modalities in the management of anterior uveitis refractory to topical treatment and intermediate or posterior uveitis, particularly unilateral cases. Systemic corticosteroids remain the initial drug of choice for most patients with severe bilateral intermediate or posterior uveitis. Therapy is initiated with 1.0 to 2.0 mg/Kg of oral prednisone or prednisolone as a single morning dose, followed by a slow taper. Use of intravenous pulse steroid therapy is an important option in acute, severe, bilateral posterior segment inflammation. In several cases, the level of systemic steroid required to control the inflammation is too high and unacceptable. Immunosuppressive drugs as steroid‐sparing agents are indicated is such cases. Intravitreal injection of triamcinolone acetonide and slow‐release intraocular devices are therapeutic options that can be used in selected uveitis cases refractory to conventional therapy and biologic agents.

Intermediate uveitis: long-term course and visual outcome

Aim:In this retrospective cohort study we investigated the long-term course and visual outcomes of intermediate uveitis (IU).Methods:We performed an institutional study of patients with IU with a follow-up of at...

Smoking As a Risk Factor for Cystoid Macular Edema Complicating Intermediate Uveitis

To describe risk factors for the presence of cystoid macular edema (CME) among patients presenting with intermediate uveitis. Cross-sectional study. Single-center, academic practice. Two hundred and eight patients with intermediate uveitis evaluated from July 1, 1984 through September 30, 2006. Clinical and demographic data were entered retrospectively into a database and analyzed. Presence of CME at presentation to our clinic; risk factors for presenting with CME. Of the 208 patients, 74% had bilateral intermediate uveitis, yielding 363 affected eyes. Eighty-nine patients (43%) had CME in at least one eye at the time of presentation to our clinic. After controlling for potentially confounding variables including demographics, duration of disease, active intraocular inflammation, history of diabetes mellitus or hypertension, and presence of epiretinal membrane, actively smoking at presentation was associated with a four-fold increased risk of CME at presentation vs never smoking (odds ratio (OR), 3.90; 95% confidence interval (CI), 1.43, 10.66; P = .008). Former smoking also appeared to increase the risk of CME at presentation in the multivariate analysis, but the result was of borderline statistical significance (OR, 1.97; 95% CI, 0.99, 3.94; P = .055). After adjusting for confounding, there was a 4% increased risk of CME at presentation for each cigarette smoked per day (OR, 1.04; 95% CI, 1.01, 1.7; P = .005). CME was a common structural ocular complication observed in our cohort. Current smoking was associated with a dose-dependent increased risk of having CME at the time of presentation to our clinic.