Enfermedad de Vogt-Koyanagi-Harada. Características de un grupo de pacientes andaluces / Vogt-Koyanagi-Harada disease. Characteristics of a series of patients

Objetivo. Describir las características de una serie de pacientes diagnosticados de enfermedad de Vogt-Koyanagi-Harada (EVKH) seguidos en una Unidad de Uveítis (multidisciplinaria, formada por oftalmólogos e internistas) en nuestro medio. Pacientes y método. Estudio retrospectivo descriptivo de 11 pacientes con EVKH (5 varones y 6 mujeres; edad media al diagnóstico: 32,6 años) seguidos entre 1980 y 2003. Resultados. Todos sufrieron panuveítis y/o desprendimiento exudativo de retina. Aparecieron alteraciones extraoculares en todos: neurológicas en el 63,7% (meningitis aséptica y/o síntomas focales), dermatológicas en el 81,8% (vitíligo, canicie, poliosis, alopecia), hipoacusia neurosensorial (el 50% de pacientes con audiometría) y síntomas generales en el 25%. Todos recibieron glucocorticoides sistémicos. Se añadió ciclosporina en 5 pacientes (45,4%) con inflamación de polo posterior y azatioprina en 2 por inflamación anterior. La agudeza visual final fue > 0,5 en el 81,8% de los casos, pero 2 tuvieron mala evolución (uno, con catarata y queratopatía en banda en ojo izquierdo y deterioro visual grave, precisó vitrectomía derecha y en otro se desarrolló hipotensión ocular grave). Aparecieron complicaciones en otros 2 pacientes: catarata y glaucoma en uno y sinequias iridianas sin glaucoma en otro. Conclusiones. Encontramos menor frecuencia de desprendimiento exudativo de retina y mayor de alteraciones dermatológicas que lo comunicado. Un porcentaje importante de casos precisó inmunosupresores. La agudeza visual final fue buena en la mayoría de los pacientes (AU)

Background. To describe the characteristics of a series of patients diagnosed of Vogt-Koyanagi-Harada disease (VKHD) and controlled by an Uveitis Unit (composed of ophthalmologistsand internists) in our population. Patients and method. Retrospective descriptive study of 11 patients with VKHD (5 males and 6 women; median age at diagnosis 32.6 years old) followed-up between 1980 and 2003. Results. All patients suffered panuveitis and/or exudative retinal detachment. Extraocular signs were present in all cases: neurological in 63.7% (aseptic meningitis and/or focal symptoms), cutaneous in 81.8% (vitiligo, whiteness, poliosis, alopecia), neurosensorial hypoacusis (50% of patients with audiometry), and general symptoms in 25%. They all received systemic corticosteroids. Cyclosporine was added in 5 patients (45.4%) with posterior uveitis, and azathioprine in 2 of them for iridocyclitis. The final visual acuity was 0.5 or better in 81.8% of cases, but 2 patients had an unfavourable evolution (one, who had cataract and band keratopathy in the left eye and severe visual worsening, needed right vitrectomy, and the other suffered severe ocular hypotension). Complications developed in 2 other patients: cataract and glaucoma in one, and synechiae without glaucoma in the other. Conclusions. We found less frequency of exudative retinal detachment and greater frequency of cutaneous signs than the communicated. A significant percentage of cases needed immunosuppressive agents. Final visual acuity was good in the majority of patients (AU)

[Vogt-Koyanagi-Harada disease. Characteristics of a series of Andalusian patients]. / Enfermedad de Vogt-Koyanagi-Harada. Características de un grupo de pacientes andaluces.

BACKGROUND: To describe the characteristics of a series of patients diagnosed of Vogt-Koyanagi-Harada disease (VKHD) and controlled by an Uveitis Unit (composed of ophthalmologists and internists) in our population. PATIENTS AND METHOD: Retrospective descriptive study of 11 patients with VKHD (5 males and 6 women; median age at diagnosis 32.6 years old) followed-up between 1980 and 2003. RESULTS: All patients suffered panuveitis and/or exudative retinal detachment. Extraocular signs were present in all cases: neurological in 63.7% (aseptic meningitis and/or focal symptoms), cutaneous in 81.8% (vitiligo, whiteness, poliosis, alopecia), neurosensorial hypoacusis (50% of patients with audiometry), and general symptoms in 25%. They all received systemic corticosteroids. Cyclosporine was added in 5 patients (45.4%) with posterior uveitis, and azathioprine in 2 of them for iridocyclitis. The final visual acuity was 0.5 or better in 81.8% of cases, but 2 patients had an unfavourable evolution (one, who had cataract and band keratopathy in the left eye and severe visual worsening, needed right vitrectomy, and the other suffered severe ocular hypotension). Complications developed in 2 other patients: cataract and glaucoma in one, and synechiae without glaucoma in the other. CONCLUSIONS: We found less frequency of exudative retinal detachment and greater frequency of cutaneous signs than the communicated. A significant percentage of cases needed immunosuppressive agents. Final visual acuity was good in the majority of patients.

[Multiple evanescent white dot syndrome. A diagnostic dilemma]. / Síndrome de múltiples puntos blancos evanescentes de difícil diagnóstico.

PURPOSE/METHOD: We present a patient with multiple evanescent white-dot syndrome (MEWDS). On presentation the patient was not initially diagnosed. The diagnosis was established eighteen months later by the clinical, angiographic and electrophysiologic features. We emphasize the difficulties to establish the diagnosis, especially in those cases that like ours present differences with regard to the initial description of the syndrome. RESULTS/CONCLUSIONS: To establish the diagnosis of MEWDS may be a difficult task because early on presentation it can be misdiagnosed. Because of this, some unnecessary tests may be performed and the patient can receive ineffective treatments and suffer possible side effects.

Síndrome de múltiples puntos blancos evanescentes de difícil diagnóstico / Multiple evanescent white dot syndrome. A diagnostic dilemma

Objetivo/Métodos: Presentamos un caso de Síndrome de múltiples puntos blancos evanescentes (SMPBE) que no fue diagnosticado inicialmente. Se confirmó al año y medio de seguimiento, por las características clínicas, angiográficas, y electrofisiológicas. Destacamos las dificultades para llegar a un diagnóstico, especialmente porque nuestro caso presenta variaciones con respecto a la descripción inicial. Resultados/Conclusiones: El Síndrome de múltiples puntos blancos evanescentes (SMPBE) es un diagnóstico difícil porque en su inicio se puede confundir con otras patologías. Esta dificultad hace que se realicen pruebas innecesarias, así como tratamientos inoperantes, en algunos casos, incluso adversos (AU)

Progressive outer retinal necrosis in an immunocompetent patient.

Progressive outer retinal necrosis syndrome is a variant of necrotizing herpetic retinopathy, a group of retinal infections caused by the herpes viruses. It has been described only in immunosuppressed patients. We present a healthy immunocompetent 16-year-old male who suffered a bilateral progressive outer retinal necrosis. Varicella-zoster virus infection was confirmed on the basis of serologic study. Treatment with intravenous acyclovir and oral prednisone was successful.

[The use of interferon alfa-2r in the treatment of autoimmune uveitis (primary or associated with Behçet's disease)]. / Utilización de interferón alfa 2r en el tratamiento de las uveítis autoinmunes (primarias o asociadas a enfermedad de Behçet).

BACKGROUND: The improvement in ocular inflammation in patients with cyclosporine A resistant Behçet disease (CyA) during therapy of severe aphthosis with recombinant interferon-alpha 2b (IFN alpha) prompted its evaluation for treatment of refractory autoimmune uveitis. METHOD: IFN alpha was used in 18 patients with posterior uveitis, nine with Behçet disease and nine with primary uveitis (mean evolution time 3.8 years) previously treated with corticosteroids (18), CyA (16) and azathioprine (1). The dose was 5 MU/day (4 weeks) and then twice weekly (12 weeks) maintaining the previous therapy. Evaluations were made at the start of the study, two weeks and two months and a cross-sectional study in February 1995. RESULTS: At the early phase both vitreal cellularity (p = 0.01) and macular edema (p = 0.003) improved; at the late phase improvements were noted in vitreal cellularity (p < 0.0001), macular (p < 0.0001) and papillar edema (p = 0.04) and visual acuity (p = 0.006). In February 1995 (mean evolution time 30 months), ten patients (56%) remain without treatment with inactive disease, six (33%) with CyA and two (11%) with CyA and IFN alpha. Improvements in vitreal cellularity (p = 0.0001), macular edema (p = 0.0001) and visual acuity (p = 0.013) were still present although macular ischemia was more severe than at the beginning (p = 0.035). The most important adverse reactions (late reactions) included depression (three cases) and thyroid changes (two cases). CONCLUSIONS: IFN alpha is an important therapeutic alternative for posterior uveitis refractory to corticosteroid therapy (included CyA).

[Serologic and molecular HLA typing in patients from Andalucia with Behçet's disease. Genetic and clinical correlations]. / Tipificación HLA serológica y molecular en pacientes andaluces con enfermedad de Behçet. Correlaciones geneticoclínicas.

BACKGROUND: HLA typing was performed in 67 patients with Behçet's disease from Andalucia, Spain to: 1) analyze the association of class I and II molecules with Behçet's disease in Spain; 2) study the clinical correlations and 3) evaluate its diagnostic and/or prognostic role. PATIENTS AND METHODS: 1) Serologic typing (microlymphocytotoxicity): class I molecules in 67 patients and class II molecules in 47 patients (controls: 223 healthy volunteers). 2) Oligotyping (PCR-SSO): class II molecules (DQB1 and generic DRB1) in 47 patients and 189 controls. 3) STATISTICAL ANALYSIS: chi square test (dicotomic variables) and Student t test (continuous variables), and calculation of the relative risk by the Wolff and Haldane formulas. RESULTS: The HLA B51 antigen was the most frequently observed in the whole series (p = 0.003) in males with ocular disease (p = 0.0001) and in patients with cutaneous (p = 0.001) and digestive involvement (p = 0.05). The HLA B51-positive males were younger at disease onset (p = 0.01) with neurologic involvement being infrequent (p = 0.03). The HLA B51 antigen was associated with neurologic (p = 0.06) and articular involvement (p = 0.05). The DQB1*0303 was associated with uveitis of bad evolution (p = 0.01). The DR11 and DQB1*0301 were more frequent in HLA B51-positive patients and the DQ5 was negatively associated with Behçet's disease, particularly in the HLA B51-positive patients. CONCLUSIONS: The study of the HLA antigens provides useful information for the diagnosis of Behçet's disease, aids in differentiating the different clinical forms and has prognostic significance.

[Type I herpes virus, HLA phenotype and Behçet disease]. / Virus herpes tipo I, fenotipo HLA y enfermedad de Behçet.

BACKGROUND: The pathogenic factors of Behçet's disease (BD) differentiate according to geographical zones. The aim of this study was to evaluate the possible relation between this process and infection by type I herpes simplex virus (HSV-1) in Spain. METHODS: The prevalence of antibodies vs HSV-1 and cytomegalovirus (CMV) was studied by complement fixation in three series: A) 34 patients with BD; B) 21 with primary uveitis; C) 40 controls. HSV-1 specificity was established by ELISA. HLA typing was performed in 32 individuals from series A. RESULTS: The frequency of anti HSV-1 in A (76%) was greater (p = 0.045) than in C (55%) and B (66%). These differences were more evident when titers equal/superior to 1/64 were considered as positive, with this being a value exceeding the 97.5% percentage of the control series (A = 21%, C = 2%, p = 0.015), specially for the ocular BD subgroup (26%, p = 0.011 with respect to C). The B series did not differ from C (p = 0.88). To the contrary no differences were observed in the distribution of titers vs CMV. Although a greater frequency of anti HSV-1 was observed in HLA-B51+ the differences were not significant (p = 0.09). CONCLUSIONS: The frequency of evaluable titers of anti-HSV-1 is greater in patients with BD (specially with ocular involvement) than in the control series. No association was found between higher titers and HLA PB51+ phenotype.