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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.

Adolescent↗

Nested polymerase chain reaction for Mycobacterium tuberculosis DNA detection in aqueous and vitreous of patients with uveitis.

BACKGROUND: Tuberculosis may be a lethal disease. Its ocular manifestations are commonly associated with severe difficulties in diagnosis and therapy; furthermore, it may cause blindness. DNA amplification methods may allow early detection of small amounts of Mycobacterium tuberculosis DNA to afford the possibility of prompt diagnosis. We evaluated a nested polymerase chain reaction (nPCR) assay for detection of Mycobacterium tuberculosis DNA in aqueous and vitreous. METHODS: In a case-control study, 22 cases of diagnosed TB uveitis (three HIV-infected patients) and 38 controls (18 HIV-infected patients) with other types of uveitis (syphilis, nine; cytomegalovirus, seven; toxoplasmosis, five; herpes simplex, one; autoimmune vasculitis, eight; Vogt-Koyanagi-Harada, four; pars planitis, one; serpinginous choroiditis, one; Wegener granulomatosis, one; and Fuchs iridocyclitis, one studied). Samples from aqueous or vitreous were cultured and analyzed by nPCR for presence of M. tuberculosis nucleic acids. We used two sets of primers corresponding to IS6110 region coding for 219 bp and 123 bp DNA sequences. RESULTS: Results were confirmed by Southern blot. All samples were tested by PCR simultaneously for Herpes simplex I, Herpes zoster, cytomegalovirus (CMV) and Toxoplasma gondii. nPCR was positive in 17 cases (77.2%) and only in three controls (8.8%) p = 0.022. All cultures were negative. Southern blot confirmed all positive nPCR tests. According to our definition of cases, there were five false negative results: two in patients with pulmonary tuberculosis; two in patients with tuberculous lymphadenitis, and one with positive skin test and hematuria. There were three cases considered false positives for nPCR: one with autoimmune vasculitis, and two with toxoplasmic uveitis. CONCLUSIONS: nPCR for TB in ocular fluids was positive in the majority of cases of ocular TB. This method is useful in early confirmation of ocular tuberculosis.

Adolescent↗

Cyclic presentation of central macular oedema.

We present the case of a young lady with unilateral pars planitis and associated cystoid macular oedema in whom visual symptoms fluctuated regularly during successive menstrual cycles. To date no such presentation of central macular oedema has been reported in the literature.

Adult↗

Fuchs' Heterochromic Uveitis: a reappraisal of the clinical spectrum.

The varying clinical appearance within a group of 103 patients with Fuchs' Heterochromic Uveitis (FHU) is discussed. Bilateral cases accounted for 7.8% of patients. Cataract was found in 80.2% of cases after 8.8 years mean follow-up, and glaucoma affected 26.2% of patients. The most important diagnostic criteria are the typical pattern of iris atrophy and depigmentation, the characteristic intraocular inflammation, and complicated cataract. These are described in detail. The correct diagnosis of FHU is important because it affects future management and prognosis. The presence or absence of macroscopic heterochromia is not a helpful component of the ocular examination and should play little part in diagnosis. FHU does not necessarily present in its classical form, and may first resemble acute anterior uveitis or pars planitis. The syndrome may be the end stage of a number of different conditions.

Adolescent↗

The spectrum of ocular toxocariasis.

Ocular toxocariasis is rare and therefore the spectrum of clinical disease is difficult to establish. We present a review of the clinical features and laboratory findings in a group of patients with positive Toxocara serology and ocular toxocariasis. The clinical spectrum was diverse and milder disease was commoner than might be supposed from reviews of the literature. Eosinophilia was unusual, but featured in two cases of unilateral pars planitis.

Adolescent↗

Steroid-induced ptosis: case studies and histopathologic analysis.

PURPOSE: The purpose of this study was to review patients who developed ptosis after subtenon's steroid injection and to study the mechanism of steroid-induced ptosis in an animal model. METHODS: Part 1. Twenty-two patients with uveitis who had received posterior subtenon's triamcinolone acetonide injections were retrospectively reviewed. Demographics, type of uveitis, type and number of surgeries, pre and postoperative marginal reflex distance (MRD1), and clinical outcomes were evaluated. Part 2. Study of rabbit levator muscle and aponeurosis histopathology after subtenon's triamcinolone injection was performed. RESULTS: Part 1. The average age was 44.6 years (range: 14-85 years) with a mean follow-up of 14 months. The most common causes of uveitis included uveitis after cataract extraction (five), pars planitis (three), multifocal choroiditis (three), and juvenile rheumatoid arthritis (three). The average time to documented onset of ptosis was 13.9 months (range: 0-49 months). In patients who received only one steroid injection, the average time to ptosis recorded was 2.7 months (range: 0-6 months). Seventeen patients underwent ptosis repair. Part 2. No ptosis was noted in the experimental and control groups. Histopathologic analysis of levator tissues revealed no significant difference in atrophy or degree of inflammation between experimental and control groups. CONCLUSIONS: Ptosis following subtenon's steroid injection ranged from mild to moderate and occurred a few months after steroid injection. Prior studies of muscles and periocular tissues exposed to corticosteroids demonstrated degenerative muscle changes; our studies revealed no histopathologic changes in the levator muscle or aponeurosis.

Adolescent↗

Clinical aspects of ocular leptospirosis in New Caledonia (South Pacific).

PURPOSE: The incidence of leptospirosis is very high in New Caledonia (average annual incidence rate: 180/100 000 of the population). To investigate the role of pathogenic leptospires as an aetiological agent of ocular diseases, we report the results of a 5-year survey in New Caledonia. METHODS: We reviewed 13 patients (corresponding to 17 investigated pathologic eyes) retrospectively. The selection of patients was based on epidemiological data, initial clinical examination, biological confirmation of leptospirosis according to reference procedures and a specific polymerase chain reaction assay. The anatomic criteria of uveitis and the criteria describing the evolution of the disease were those recommended by the International Uveitis Study Group. RESULTS: Consequent rates of optic neuritis (65%), posterior uveitis (35%), secluded pupil (24%), interstitial keratitis (18%) and pars planitis (12%) were found. Our rates of recurrence (46%) and of ophthalmic complications (82%) were substantial; some symptoms indicated brain involvement.The final visual damage was severe in 35% of eyes. CONCLUSIONS: Microagglutination and polymerase chain reaction hybridization are complementary tests for the diagnosis of Leptospira-induced ophthalmic lesions. Before deciding on treatment, one must consider the ability of virulent leptospires to persist in immunologically privileged sites such as aqueous humor, cerebrospinal fluid and eukaryotic cells. Polymerase chain reaction is a useful tool for the diagnosis of Leptospira-induced ocular complications, which are probably misdiagnosed if based only on routine laboratory tests. It enables early diagnosis and early specific treatment that now consists of quinolone and cyclines.

Adult↗

[Incidence of ocular manifestations in patients with histologically confirmed systemic sarcoidosis].

BACKGROUND: Intraocular findings in sarcoidosis were described by US studies with 35 % anterior uveitis, 25 % posterior uveitis and 20 - 30 % for late complications in case of first presentation. We report on the spectrum and diagnostic possibilities in 38 German patients. PATIENTS AND METHODS: 38 patients (68 eyes) aged 11 - 58 years (average 42.7 y), who suffered of intraocular manifestations in histological confirmed systemic sarcoidosis (1987 - 1997), were completely evaluated retrospectively. For additional diagnostic tools fluorescein angiography, optical coherence tomography, nuclear magnetic resonance tomography and measurements of angiotensin-converting enzyme, lysozyme and calcium in serum were performed. RESULTS: 15 patients (22 eyes = 32.4 %) had anterior uveitis: granulomatous keratouveitis (10 eyes), granulomas of the iris (6), granulomas of the trabecular meshwork (3) and 22 patients (42 eyes = 61.8 %) an intermediate and posterior uveitis: granulomatous perivasculitis (18 eyes), cystoid maculopathy (8), pars planitis (6), neuroretinitis (4), optic neuritis (3), choroiditis (3). Late complications in case of first presentation were found in 22 patients (39 eyes = 57.4 %): posterior synechiae 14 x, secondary glaucoma 10 x, cataracta complicata 8 x, optic atrophy 6 x, intraocular calcification 1 x. CONCLUSION: Compared to previous studies with heterogeneous ethnic composition, intraocular inflammations with confirmed sarcoidosis in German patients show more than 60 % involvement of the posterior segment. We may speculate on the reasons for these discrepancies as being due to ethnic reasons, age and long-term follow-up. In case of typical ocular signs of sarcoidosis, treatment should be started immediately even without histological confirmation. In some cases histological confirmation is successful 8 years after first presentation.

Adolescent↗

[Uveitis in the elderly].

BACKGROUND: There is only scarce information available in the literature on uveitis of older patients. The aim of this study was to study this age group in greater detail. PATIENTS AND METHODS: 435 patients were seen in the uveitis clinic of the University Eye Hospital Jules Gonin from January 1990 to March 1993. We analysed here the 94 (21.8%) patients that presented a first episode of uveitis after the age of sixty. RESULTS: This collective was characterised by an increased frequency of the anatomic diagnosis of panuveitis (p < 0.0001), and of the specific diagnosis of zoster uveitis (p < 0.0001), sarcoidosis (p < 0.0001), uveitis associated with scleritis (p < 0.05) and granulomatous anterior uveitis (p < 0.01). The frequency of diagnosis of intermediate uveitis was significantly reduced (p < 0.034) as were the specific diagnosis of HLA-B27 positive acute anterior uveitis (p < 0.001), pars planitis (p < 0.08) and non granulomatous anterior uveitis (p < 0.01). Reiter's uveitis, Possner-Schlossmann Syndrome, Behçet's uveitis, Vogt-Koyanagi-Harada disease and Toxoplasmosis retinochoroiditis were never diagnosed in this group of elderly patients. The proportion of defined specific diagnosis (75.5%) was comparable to the group of patients of less than 60 years old. CONCLUSION: As can be seen from our data, epidemiological characteristics of old age uveitis differ markedly from younger patients which should influence the diagnostic approach in these patients.

Aged↗

[Ocular involvement by Epstein-Barr virus: a difficult diagnosis].

BACKGROUND: A correlation has been made, often on the base of very scarce evidence, between several ocular clinical pictures and chronic infection due to the Epstein-Barr virus (EBV). MATERIALS AND METHODS: Serology of the herpes group viruses is part of the investigations performed in uveitis cases without obvious etiology that are examined at our uveitis clinic. We retrospectively studied the clinical files of patients with an elevated EBV serology for the purpose of studying the type of ocular pathology presented. RESULTS: According to our criteria, 40 patients had an elevated EBV serology. Twenty-nine presented with a polyclonal elevation of antibodies against several viruses of the herpes group. Six showed an increase of anti-EBV antibodies only (2 uveitis cases without specific diagnosis, 2 HLA-B27 positive anterior uveitis cases, 1 pars planitis and 1 anterior uveitis with endothelitis). CONCLUSIONS: If we exclude the ocular pathology occurring during acute primary infection (infections mononucleosis), it is difficult to find some correlation between a clinical picture and EBV infection. Before better diagnostic tools are available, the spectrum of ocular EBV disease will remain conjectural.

Antibodies, Viral↗

[Juvenile uveitis].

Chronic anterior uveitis in children suffering from juvenile rheumatoid arthritis may develop very early, when the patient is as young as two or three years old; it affects twice as many girls as boys. The arthritis is often localized in one joint (pauciarticular or oligoarticular form); Still's syndrome is very rare. Local treatment with cycloplegics and steroids has to be combined with systemic immunosuppressive medication with cyclophosphamide. Chronic intermediate uveitis (pars planitis) usually affects teenagers in good health. Peripheral fundus changes can be detected most easily by biomicroscopy with the three-mirror contact lens. Cystoid macular edema is frequent and, together with vitreous opacities, is responsible for a deterioration in central vision. In most cases local treatment with subconjunctival injection of depot steroids is sufficient. Immunosuppressive treatment or surgical intervention is only indicated in severe cases where there is a risk of complete loss of vision.

Arthritis, Juvenile↗

Seropositivity of human herpesvirus-8 in patients with uveitis.

PURPOSE: To explore the role of human herpesvirus-8 (HHV-8) in the pathogenesis of uveitis and other forms of ocular inflammation. METHODS: Serum antibody titers to HHV-8 were measured in 76 patients with uveitis from various causes and other types of ocular inflammation in either the acute phase, the convalescent phase, or both. RESULTS: Only one 21-year-old male patient in the convalescent phase of unilateral pars planitis showed a positive titer for HHV-8. His serum was negative for human immunodeficiency virus-1. CONCLUSIONS: Although the seropositive rate in the patient population was higher than that in the general population, the increased incidence was not statistically significant. The role of HHV-8 in the pathogenesis of intraocular inflammation appears to be limited.

Adolescent↗

Uveitis referral pattern in a Midwest University Eye Center.

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

Academic Medical Centers↗

Combined orbital floor betamethasone and depot methylprednisolone in uveitis.

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

Adolescent↗

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

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

Adolescent↗

Ocular manifestations of cat-scratch disease.

Bartonella henselae has only recently been isolated, characterized, and found to be the principal cause of cat-scratch disease (CSD). The availability of specific serologic investigations has allowed the recognition of a spectrum of ocular CSD syndromes that previously were ill defined and considered idiopathic. The primary inoculation complex causing regional lymphadenopathy is represented in the eye by Parinaud's oculoglandular syndrome; B. henselae is the most common cause. Leber's neuroretinitis has been identified for 80 years, and new data suggest that it is commonly a manifestation of CSD; the extent of the association remains to be determined. CSD optic neuritis is also described. The vitreoretinal manifestations include anterior uveitis, vitritis, pars planitis, focal retinal vasculitis, a characteristic retinal white spot syndrome, Bartonella retinitis, branch retinal arteriolar or venular occlusions, focal choroiditis, serous retinal detachments, and peripapillary angiomatous lesions. The pattern of ocular disease in AIDS-associated B. henselae infections is poorly delineated; unusual manifestations include conjunctival and retinal bacillary angiomatosis. The benefit of antimicrobial therapy for CSD in immunocompetent individuals has been difficult to establish, partly because most infections are self limited. Empirically, azithromycin, ciprofloxacin, rifampin, parenteral gentamicin, or trimethoprim-sulfamethoxazole provide the best therapeutic choices to minimize damage to the eye.

Anti-Bacterial Agents↗

Ocular manifestations of multiple sclerosis.

PURPOSE OF REVIEW: Multiple sclerosis is an autoimmune demyelinating disorder of the nervous system that is commonly manifested by visual system involvement and that may initially present with ophthalmologic symptoms. This paper reviews recent findings regarding the ocular manifestations in multiple sclerosis. RECENT FINDINGS: Manifestations of multiple sclerosis in the eye include both the afferent and efferent visual pathways. Optic neuritis, the most common ocular manifestation of multiple sclerosis, may be the initial clinical disease manifestation. Recent long-term follow-up data show that most patients with demyelinating optic neuritis have an excellent prognosis for recovery of central visual acuity. Evidence is emerging, however, for significant and broad reduction in both contrast sensitivity and color perception in multiple sclerosis patients despite near-normal visual acuities. Ocular motor deficits in multiple sclerosis include internuclear ophthalmoplegia and nystagmus, resulting in diplopia, oscillopsia, blurred visual, loss of stereopsis, and reading fatigue. Multiple sclerosis also may be associated with ocular inflammatory diseases, in particular pars planitis and retinal periphlebitis. SUMMARY: Ocular findings may be initial manifestations of multiple sclerosis and may predict additional demyelinating events. Recognizing these syndromes and signs will help clinicians to properly evaluate the patient, formulate an appropriate differential diagnosis, be able to discuss the prognosis with the patient, and help develop an effective therapeutic plan.

Eye Diseases↗

Uveitis: aetiology and disease associations in an Australian population.

Over a five-year period 245 patients with uveitis were investigated at the Uveitis Clinic, Sydney Eye Hospital, for possible aetiological and relevant disease associations. Uveitis was anterior in 75% of patients, posterior in 21% and generalized in 4%. Anterior uveitis (AU) was idiopathic in 52% of cases. In patients tested for the HLA-B27 antigen, 47% were HLA-B27 positive, including all cases of ankylosing spondylitis (8% of cases) and Reiter's syndrome (3% of cases). There was a marked male predominance in patients with AU, especially in HLA-B27 positive individuals. Posterior uveitis (PU) was most frequently unilateral, chronic and idiopathic (24% of cases), whilst recognizable aetiologies included toxoplasmosis (20%), Behcet's syndrome (14%), sarcoidosis (12%) and pars planitis (12%). The peak age of onset in patients presenting with AU was 30 to 40 years, whilst patients with PU presented a decade earlier. There were no major differences between males and females in the age of onset of their uveitis.

Adult↗