Esclerodermatomiositis con manifestaciones oculares predominantes / Sclerodermatomyositis, ocular manifestations

Antecedentes: La esclerodermatomiositis es un síndrome de superposición que tiene manifestaciones dermatológicas, musculares y articulares, y que puede presentar afección ocular. Caso clínico: Se presenta el caso de una mujer de 57 años en quien la exploración oftalmológica hizo evidente adelgazamiento escleral 360 grados, celularidad anterior y vítrea. La exploración física orientada y los estudios de laboratorio permitieron sustentar el diagnóstico de esclerodermatomiositis, por lo que se trató de forma sistémica. Conclusión: La esclerodermatomiositis es una enfermedad rara, cuyo diagnóstico implica estudio clínico y de laboratorio, y su manejo debe ser multidisciplinario, donde las manifestaciones oculares inflamatorias pueden estar presentes (AU)

Background: Sclerodermatomyositis is an overlap syndrome of myositis and scleroderma, with dermatological, muscular and joint involvement, but may also present with ocular manifestations. Clinical case: A 57 year-old woman presented with ophthalmological manifestations, including scleral thinning 360°, and the presence of cells in the anterior and posterior chamber. Oriented physical examination and laboratory studies led to the diagnosis, with the need for systemic treatment. Conclusion: Sclerodermatomyositis is a rare disease. Its diagnosis needs thorough clinical and laboratory studies, and its management should be multidisciplinary when inflammatory ocular manifestations may be present (AU)

Sclerodermatomyositis, ocular manifestations. / Esclerodermatomiositis con manifestaciones oculares predominantes.

BACKGROUND: Sclerodermatomyositis is an overlap syndrome of myositis and scleroderma, with dermatological, muscular and joint involvement, but may also present with ocular manifestations. CLINICAL CASE: A 57 year-old woman presented with ophthalmological manifestations, including scleral thinning 360°, and the presence of cells in the anterior and posterior chamber. Oriented physical examination and laboratory studies led to the diagnosis, with the need for systemic treatment. CONCLUSION: Sclerodermatomyositis is a rare disease. Its diagnosis needs thorough clinical and laboratory studies, and its management should be multidisciplinary when inflammatory ocular manifestations may be present.

Análisis ultrabiomicroscópico de quistes iridianos / Ultrasound biomicroscopic analysis of iris cysts

OBJETIVO: Describir los hallazgos ultrabiomicroscópicos y complicaciones de pacientes con quistes iridianos. DISEÑO: Serie de casos, restrospectivo. MÉTODO: Se incluyó a 13 pacientes con diagnóstico de quistes de iris, confirmado mediante ultrabiomicroscopia (UBM) en un periodo de 10 años (2002-2012) en un centro oftalmológico de la ciudad de México. Se incluyeron datos demográficos, historia clínica médica y ocular, características clínicas y ultrabiomicroscópicas (tipo, número, localización y hallazgos acústicos), así como complicaciones asociadas. Se realizó un análisis descriptivo, incluyendo medias y desviación estándar. RESULTADOS: La distribución por sexo fue 8 mujeres y 5 hombres, con edad promedio de 44,5 años ± 15,5 (rango de 6 a 70 años). El 92,3% fueron quistes del epitelio pigmentado y 7,7% del estroma; el 76,9% se encontraron en la periferia y 69,2% entre los meridianos de las II y las VI horas del reloj. Todos los quistes mostraron una pared con reflectividad moderada a alta. El 38,5% presentó complicaciones (el 15,4% cierre parcial del ángulo camerular; el 15,4% glaucoma secundario de ángulo cerrado y el 7,7% discoria). CONCLUSIONES: La mayoría de los quistes de iris son derivados del epitelio pigmentado, de curso benigno y con una baja tasa de complicaciones. La UBM es una herramienta indispensable que nos permite planear tratamientos localizados, específicos, más conservadores y menos destructivos, con un daño potencial menor de las estructuras oculares y, por lo tanto, mejor pronóstico visual

PURPOSE: To describe the ultrasound biomicroscopic (UBM) features and complications associated with iris cysts. DESIGN: A retrospective case series. METHODS: Thirteen patients with iris cysts were identified in a 10 year period study at a ophthalmologic reference Center in Mexico City. The variables included demographic data, ocular and medical history, clinical course, and complications. All patients were examined by UBM, and type, number, location, and acoustic characteristics of cysts were evaluated. Descriptive statistics were performed. RESULTS: Thirteen patients were included (8 men and 5 women). The mean age was 44.5 ± 15.5 years (range 6-70 years). The origin most prevalent was neuroepithelial (92.3%), and 7.7% had stromal cysts. Regarding to location 76.9% were found in the periphery, and 69.2% between meridians II and VI. All cysts showed a moderate to high reflectivity in the wall. Complications were present in 38.5% of cases (15.4% partial angle closure, 15.4% secondary angle closure glaucoma and 7.7% dyscoria). CONCLUSIONS: Most cysts are derived from iris pigmented epithelium, with a benign course and a minor rate of complications. The UBM is an indispensable tool that allows us to plan more specific and conservative treatments, with less damage to ocular structures and, therefore, better visual prognosis

Ultrasound biomicroscopic analysis of iris cysts.

PURPOSE: To describe the ultrasound biomicroscopic (UBM) features and complications associated with iris cysts. DESIGN: A retrospective case series. METHODS: Thirteen patients with iris cysts were identified in a 10 year period study at a ophthalmologic reference Center in Mexico City. The variables included demographic data, ocular and medical history, clinical course, and complications. All patients were examined by UBM, and type, number, location, and acoustic characteristics of cysts were evaluated. Descriptive statistics were performed. RESULTS: Thirteen patients were included (8 men and 5 women). The mean age was 44.5 ± 15.5 years (range 6-70 years). The origin most prevalent was neuroepithelial (92.3%), and 7.7% had stromal cysts. Regarding to location 76.9% were found in the periphery, and 69.2% between meridians II and VI. All cysts showed a moderate to high reflectivity in the wall. Complications were present in 38.5% of cases (15.4% partial angle closure, 15.4% secondary angle closure glaucoma and 7.7% dyscoria). CONCLUSIONS: Most cysts are derived from iris pigmented epithelium, with a benign course and a minor rate of complications. The UBM is an indispensable tool that allows us to plan more specific and conservative treatments, with less damage to ocular structures and, therefore, better visual prognosis.

Pacientes con pars planitis tratados con fotocoagulación selectiva: curso clínico / Clinical course of pars planitis in patients treated with selective photocoagulation

Introducción: La pars planitis es una uveítis intermedia, con una presentación bilateral y asimétrica. La etiología es desconocida y la patogenia no está clara. El tratamiento sigue el algoritmo de Foster, donde se incluye la fotocoagulación selectiva. El mecanismo de acción de la fotocoagulación es desconocido. Materiales y métodos: Estudio observacional, longitudinal, cohorte ambilectivo. El objetivo fue evaluar el curso de la inflamación en pacientes con pars planitis tratados con láser argón selectivo. Resultados: Se incluyó a 29 pacientes con diagnóstico de pars planitis tratados con láser selectivo; 10 pacientes eran del género femenino y 19 del masculino. La edad promedio de inicio de la enfermedad fue 11,37 años. De los pacientes, en el momento de recibir el láser selectivo, 18 (62,1%) no se encontraban con inmunosupresión y 11 (37,9%) estaban con inmunosupresor. Las indicaciones de láser fueron por: seguimiento del algoritmo 19 (65,55%), hemorragia vítrea siete (24,1%), vitrectomía dos (6,98%) y neovasos uno (3,4%). El tiempo promedio de disminución de la inflamación fue de 5,9 meses y 17 pacientes (58,6%) no presentaron recaída. Las agudezas visuales presentaron mejoría posláser (OD p = 0,025 y OI p = 0,022). Se observó mejoría clínica de la celularidad vítrea. Conclusión: El láser selectivo demostró ser efectivo en el 58,6%% de los pacientes (AU)

Introduction: Pars planitis is an intermediate uveitis with bilateral and asymmetric presentation. The etiology is unknown and pathogenesis is unclear. Treatment follows the algorithm of Foster, which includes selective photocoagulation. The mechanism of action of photocoagulation is still unknown. Material and methods: An observational, longitudinal, ambispective cohort study was performed with the objective of evaluating the course of inflammation in patients with pars planitis treated with a selective argon laser. Results: The study included 29 patients (10 female and 19 male) diagnosed with pars planitis and were treated with selective laser. The mean age of onset was 11.77 years. Eighteen (62.1%) patients were not immunosuppressed at the time of receiving the selective laser, and 11 (37.9%) were taking immunosuppressants. Indications for selective laser were; following the algorithm, 19 (65.55%), vitreous hemorrhage 7 (24.1%), vitrectomy 2 (6.98%), and neovascularization 1 (3.4%). The mean time for inflammation reduction was 5.9 months, and 17 patients (58.6%) had no relapse. Visual acuity showed improvement post-laser (OD P = 0.025 and OI P = 0.022). There was also an improvement in vitreous cells. Conclusion: Selective laser was effective in 58.6%% of patients (AU)

[Clinical course of pars planitis in patients treated with selective photocoagulation]. / Pacientes con pars planitis tratados con fotocoagulación selectiva: curso clínico.

INTRODUCTION: Pars planitis is an intermediate uveitis with bilateral and asymmetric presentation. The etiology is unknown and pathogenesis is unclear. Treatment follows the algorithm of Foster, which includes selective photocoagulation. The mechanism of action of photocoagulation is still unknown. MATERIAL AND METHODS: An observational, longitudinal, ambispective cohort study was performed with the objective of evaluating the course of inflammation in patients with pars planitis treated with a selective argon laser. RESULTS: The study included 29 patients (10 female and 19 male) diagnosed with pars planitis and were treated with selective laser. The mean age of onset was 11.77 years. Eighteen (62.1%) patients were not immunosuppressed at the time of receiving the selective laser, and 11 (37.9%) were taking immunosuppressants. Indications for selective laser were; following the algorithm, 19 (65.55%), vitreous hemorrhage 7 (24.1%), vitrectomy 2 (6.98%), and neovascularization 1 (3.4%). The mean time for inflammation reduction was 5.9 months, and 17 patients (58.6%) had no relapse. Visual acuity showed improvement post-laser (OD P=.025 and OI P=.022). There was also an improvement in vitreous cells. CONCLUSION: Selective laser was effective in 58.6%% of patients.

[Current immunologic and molecular data on the epidemiology of histoplasmosis in Morelos, Mexico]. / Actualidades inmunológicas y moleculares sobre la epidemiología de la histoplasmosis en Morelos, México.

The aim of the present paper was to analyze current data distribution of systemic mycosis, i.e. histoplasmosis, in the state of Morelos, Mexico. Data were collected based on immunoepidemiologic studies and preliminary molecular-epidemiologic results. The occupational activities of the rural population exposed to risk of infection, findings on genetic polymorphisms, and spread of the causative agent in nature were considered. These will be processed to propose criteria for establishing the prevalence of this mycosis in Morelos and to elaborate an epidemiologic map of the state.

Secondary glaucoma in patients with uveitis.

PURPOSE: To evaluate the prevalence of secondary glaucoma (SG), clinical forms of uveitis more frequently associated with glaucoma, and describe the treatment and complications encountered in a cohort of patients with glaucoma and uveitis during a 10-year period. METHODS: The hospital records of patients with uveitis referred to the Immunology Service of the Massachusetts Eye and Ear Infirmary for a decade were reviewed for cases of SG. RESULTS: One hundred and twenty of the 1,254 patients (9.6%) with uveitis developed SG. SG was more frequent in anterior uveitis (67%) but was also associated with posterior uveitis (13%) and pars planitis (4%). Herpetic keratouveitis (22%), Fuchs' iridocyclitis (19%), juvenile rheumatoid arthritis-associated iridocyclitis (16%), syphilis (14%), and sarcoidosis (12%) were the leading types of uveitis associated with SG. Despite aggressive medical and surgical therapy, SG was associated with progressive visual field loss and optic nerve damage in 39 patients (33%). CONCLUSION: SG is an underappreciated, vision-threatening complication in patients with uveitis. Increased vigilance for emergence of this complicating problem during the care of patients with uveitis is warranted, and medical and surgical treatment for reducing IOP should be especially aggressive in these patients. We hypothesize that earlier, more aggressive treatment of uveitis will reduce the presence of glaucoma as an additional vision-robbing complication of uveitis.

Outcomes in anterior uveitis associated with the HLA-B27 haplotype.

OBJECTIVE: This study aimed to test the hypothesis that patients presenting with anterior uveitis who are HLA-B27 positive, either with or without associated systemic disease, have a less-favorable outcome than do patients with idiopathic anterior uveitis who are HLA-B27 negative. DESIGN: Retrospective case-controlled series. PARTICIPANTS: Ninety-seven patients who were HLA-B27 positive with no systemic disease, 94 patients who were HLA-B27 positive with systemic disease, and 72 patients who were HLA-B27 negative who presented with anterior uveitis were studied. MAIN OUTCOME MEASURES: Ocular complications (e.g., secondary glaucoma, cataract formation, pupillary synechiae, vitritis, cystoid macular edema, and optic disc edema), medical and surgical treatment, number of recurrent attacks, and final visual acuity were recorded for all patients. RESULTS: The patients who were HLA-B27 positive, either with or without systemic disease, experienced a greater number of complications than did the patients who were HLA-B27 negative. Periocular corticosteroids, systemic corticosteroids, and systemic immunosuppressive chemotherapy were required in a far greater number of HLA-B27-positive patients than in HLA-B27-negative patients (60% vs. 11%, 53% vs. 7%, and 18% vs. 1%, respectively; P < 0.001). The percentage of legally blind eyes was significantly greater in the HLA-B27-positive group, both with and without systemic disease, when compared with the HLA-B27-negative group (11% vs. 2%; P < 0.005). CONCLUSIONS: The prognosis of anterior uveitis associated with the HLA-B27 haplotype, either with or without associated systemic disease, is less favorable when compared with that of HLA-B27-negative patients with idiopathic anterior uveitis.

Referral patterns of uveitis in a tertiary eye care center.

OBJECTIVE: To analyze the referral patterns and diagnosis of uveitis during the past decade in a large tertiary eye center. DESIGN: The records of 1237 patients with uveitis referred to the Immunology Service of the Massachusetts Eye and Ear Infirmary from 1982 to 1992 were classified and analyzed. Data regarding sex, race, nationality, referral site, ages at presentation and onset of uveitis, ocular involvement, clinical characteristics, ocular condition, and systemic disease associations were obtained. RESULTS: The mean age at onset of uveitis was 37.2 years; the male-to-female ratio was 1:1.4. Most patients were white (85.8%), born in the United States (83.1%), and referred from within New England (84.7%). Anterior uveitis was most common (51.6%), followed by posterior uveitis (19.4%), panuveitis (16.0%), and intermediate uveitis (13.0%). Chronic (58.3%), nongranulomatous (77.7%), and noninfectious (83.1%) were the most frequent types of uveitis. The most common entities included idiopathic (34.9%), seronegative spondyloarthropathies (10.4%), sarcoidosis (9.6%), juvenile rheumatoid arthritis (5.6%), systemic lupus erythematosus (4.8%), Behçet's disease (2.5%), and the acquired immunodeficiency syndrome (2.4%). CONCLUSION: The appearance of new uveitic entities, such as the acute retinal necrosis syndrome, multifocal choroiditis and panuveitis, birdshot retinochoroidopathy, and acquired immunodeficiency syndrome-related uveitis, and the reemergence of the classic infectious causes of uveitis, tuberculosis and syphilis, have changed the way we approach the diagnosis and management of posterior and panuveitis at the Massachusetts Eye and Ear Infirmary.

The value of combined serum angiotensin-converting enzyme and gallium scan in diagnosing ocular sarcoidosis.

PURPOSE: To evaluate the role of combined serum angiotensin-converting enzyme (ACE) activity and whole-body gallium (67GA) scanning in diagnosing sarcoidosis in patients with features consistent with ocular sarcoidosis but with normal or equivocal chest radiographs. METHODS: Serum ACE levels and whole-body 67GA scans were obtained as part of the initial workup in 22 patients with active ocular inflammation and ultimately biopsy-proven sarcoidosis (sarcoid uveitis group). A second group consisting of 70 patients with active uveitis in whom sarcoidosis also was considered a diagnostic possibility also was studied. All 70 patients ultimately had a definitive diagnosis other than sarcoidosis (nonsarcoid uveitis). All patients in this group also had a serum ACE and whole-body 67GA scan performed as part of their initial investigations. RESULTS: All patients in the sarcoid uveitis group had either an elevated ACE level or an abnormal scan. In 16 of the 22 patients, results of both tests were abnormal. In no patient in the nonsarcoid uveitis group were results of both tests abnormal. The sensitivity of an elevated ACE in diagnosing sarcoidosis was 73% and the specificity was 83%. Using the combination of a positive 67GA scan and an elevated ACE, the specificity for diagnosis was 100% and the sensitivity was 73%. CONCLUSIONS: The combination of serum ACE level and whole-body 67GA scan increases the diagnostic specificity without affecting sensitivity in patients with clinically suspicious ocular sarcoidosis who have normal or equivocal chest radiographs.

Systemic immunosuppressive therapy and the occurrence of malignancy in patients with ocular inflammatory disease.

PURPOSE: To compare the occurrence of malignancy in patients with severe ocular inflammatory disease treated with systemic corticosteroids alone or with systemic immunosuppressive drugs with or without systemic corticosteroids. METHODS: The clinical records of 543 patients with ocular inflammatory disease treated with systemic corticosteroids and/or immunosuppressive chemotherapy were reviewed in a retrospective cohort study. Characteristics of patients treated with corticosteroids alone were compared with characteristics of patients treated with immunosuppressive agents with or without preceding corticosteroid treatment. The rates of malignancy after initiation of drug therapy were compared using an exact test for incidence rate data. RESULTS: Compared with patients treated with corticosteroids alone, patients treated with immunosuppressants with or without corticosteroid treatment were older and had more severe systemic disease. During a total of 1261 person-years of follow-up, a malignancy developed in five patients. The rate of malignancy in the immunosuppressant group (4 malignancies during 968 person-years of follow-up) was not significantly different from the rate in the corticosteroids alone group (1 malignancy during 293 person-years of follow-up) (P > 0.90, exact test for incidence rate data). CONCLUSION: These findings do not support the hypothesis of an increased risk of malignancy in patients with severe ocular inflammatory disease who are treated with systemic immunosuppressive agents compared with patients treated with systemic corticosteroids.

T cell receptor V beta gene expression in experimental herpes stromal keratitis.

Our study examined T cell receptor (TCR) V beta mRNA expression in a murine model of experimental herpes simplex keratitis (HSK). We employed a polymerase chain reaction (PCR) technique to detect TCR V beta mRNA expression in the inoculated eyes of both HSK-susceptible and HSK-resistant mice at different time points after corneal inoculation with herpes simplex virus type 1 (HSV-1), followed by Southern blotting and densitometry analysis. In eyes from HSK-susceptible C.AL-20 mice, a more diverse TCR V beta transcript usage pattern was detected as compared with that seen in HSK-resistant C.B-17 mice. V beta 8 family members were expressed in both strains of mice at days 11, 14 and 21 post-inoculation. By densitometry, at day 11, the intensity of expression of V beta 8.2 and V beta 8.3 message was significantly greater in the eyes of C.AL-20 mice; V beta 8.1 was expressed only in C.B-17 mice. There were obvious differences in the TCR V beta expression between HSK-susceptible and HSK-resistant mice. The differences in the intensity of the message expressed by V beta 8 family members between the two strains could be correlated to previous experiments that showed V beta 8.1,2+ T cells as the main infiltrating cells in the corneas of HSK-susceptible mice by day 11 and 14 after challenge with HSV-1.

Differential expression of alternatively spliced fibronectin in normal and wounded rat corneal stroma versus epithelium.

PURPOSE: The polymerase chain reaction was used to examine fibronectin (FN) expression during corneal scrape wounding with specific attention to the presence, absence, or gross changes of alternatively spliced FN as differentially expressed in the corneal stroma versus the epithelium in normal and wounded tissue. METHODS: Specific FN cDNA sequences were synthesized from rat cornea with total RNA and were amplified using various sets of synthetic oligonucleotide primers. RESULTS: The authors observed the presence and sustained the expression of total FN, EIIIA, EIIIB, and V-region FN mRNA in normal and injured corneal stroma for up to 3 weeks after scrape wounding. In contrast, complementary overlying epithelial samples were virtually devoid of FN message. CONCLUSIONS: These data suggest that functionally different, alternatively spliced FN isoforms may be involved both in the maintenance of the normal cornea and in wound healing, and that their synthesis occurs in situ principally by the stroma rather than by the epithelium.

Posterior segment ocular manifestations in patients with HLA-B27-associated uveitis.

PURPOSE: To describe a series of patients with seronegative arthritic syndromes and HLA-B27-associated uveitis with severe, sight-threatening, posterior segment ocular manifestations. METHODS: The authors reviewed the records of 29 patients (17.4%) with posterior segment involvement from a cohort of 166 patients with HLA-B27-associated uveitis. The inclusion criteria included individuals with a positive HLA-B27 who had at least one of the following findings: (1) severe vitreous inflammation; (2) papillitis; (3) retinal vasculopathy; or (4) pars plana exudates. The study population comprised 13 men and 16 women with a mean age at onset of uveitis of 35.2 years. The average duration of the uveitis was 5.3 years, and the median follow-up time was 26 months. FINDINGS: Posterior segment involvement occurred in 34 eyes of the 29 patients. The most common findings included severe and diffuse vitritis in 93.1% of the patients and papillitis in 24 patients (82.7%). Retinal vasculitis occurred in seven patients (24.1%), and pars plana exudates were present in two patients (6.8%). Cystoid macular edema (37.9%) and epiretinal membrane (17.2%) were common causes of visual impairment. Systemic immunosuppressive therapy was required for control of inflammation in 32% of the patients. CONCLUSION: HLA-B27-associated uveitis may be related to severe, sight-threatening posterior segment manifestations in some patients; this is an under-recognized phenomenon. These patients may require the use of aggressive systemic immunosuppressive therapy to control inflammation and preserve vision.

The syndrome of presumed ocular histoplasmosis in Mexico: a preliminary study.

A study to screen for the syndrome of presumed ocular histoplasmosis (SPOH) among native populations from three Mexican states was performed. Two of these states, Guerrero and Querétaro, were selected as histoplasmosis is endemic there, whereas Tlaxcala was considered a control, due to the absence of reported cases. A total of 253 individuals were submitted to ocular fundus examination to obtain evidence of SPOH. A high percentage of positive reactors to histoplasmin skin test (ST) was observed in Guerrero (83%) and Querétaro (53%), whereas in Tlaxcala positive ST were almost absent (2.04%). Only five individuals had retinal lesions, although these lesions were not characteristic of the syndrome. Stimulation of these individual's cells showed different patterns in the histoplasmin-induced lymphocyte transformation response, and two out of five individuals with retinal lesions presented a stimulated response, as well as three controls without lesions. Histocompatibility antigens (HLA) were determined in a sample of each population and no particular allele, including HLA-B7, was found to be related to SPOH as reported in the USA; however, HLA-B22 was found in three individuals who developed pulmonary histoplasmosis. Results do not provide clinical evidence or data on specific HLA risk factors, for the presence of SPOH in the population studied.

Retrospective serological study of histoplasmosis in Mexico.

Mycotic immunodiagnosis was performed in 325 patients with clinical evidence of systemic mycoses, over a 5-year period, from different hospitals of Mexico City. Results showed 168 individuals that presented one positive serological test to Histoplasma capsulatum antigens. From these, only 27 patients were serologically positive to two or more tests, such as tube precipitin, immunodiffusion, complement fixation, and ELISA, and developed signs and symptoms of a histoplasmosis clinically classified as primary pulmonary. Four of them presented an underlying disease including one positive HIV patient. Twenty-two came from endemic histoplasmosis zones of the country and most of them acquired the disease in caves or uninhabited houses. The diagnosis of histoplasmosis should be based on reliable laboratory data which could raise more significant information of its incidence in Mexico.