Retinopatía autoinmune asociada a la presencia de anticuerpos onconeuronales: a propósito de un caso / Autoimmune retinopathy with onconeuronal antibodies: Case report

Se presenta el caso de retinopatía autoinmune en un paciente con carcinoma microcítico de pulmón, no conocido hasta el momento, que se diagnosticó tras la exploración oftalmológica. La serología fue positiva para anticuerpos onconeuronales CV2/CRMP5. La retinopatía autoinmune es una entidad rara que puede pasarse por alto, y ser infradiagnosticada. Se produce por una reacción inmunomediada contra antígenos retinianos. La importancia de su diagnóstico precoz radica en que en muchos de los pacientes la sintomatología ocular aparece antes del diagnóstico del cáncer primario, por lo que su identificación y derivación precoz para estudio de extensión puede suponer el diagnóstico de una neoplasia primaria oculta hasta el momento. (AU)

We present a case of autoimmune retinopathy in a patient with unknown small cell lung cáncer (SCLC), which was diagnosed after ophthalmological examination. Serology was positive for CV2/CRMP5 onconeuronal antibodies. Autoimmune retinopathy is a rare entity that can be missed and underdiagnosed. It is produced by an immune-mediated reaction against retinal antigens. The importance of its early diagnosis lies in the fact that in many of the patients, ocular symptoms appear before the diagnosis of the primary cancer, so its early identification and referral for an extension study may lead to the diagnosis of a hidden primary neoplasm. (AU)

Autoimmune retinopathy with onconeuronal antibodies: Case report.

We present a case of autoimmune retinopathy in a patient with unknown small cell lung cáncer (SCLC), which was diagnosed after ophthalmological examination. Serology was positive for CV2/CRMP5 onconeuronal antibodies. Autoimmune retinopathy is a rare entity that can be missed and underdiagnosed. It is produced by an immune-mediated reaction against retinal antigens. The importance of its early diagnosis lies in the fact that in many of the patients, ocular symptoms appear before the diagnosis of the primary cancer, so its early identification and referral for an extension study may lead to the diagnosis of a hidden primary neoplasm.

Acute panendothelial retinal leakage in a patient with diabetes mellitus type 1 with a poor metabolic control.

Ophthalmological diabetic complications are one of the main causes of blindness worldwide, so careful diagnostic and management is important. The screening plans implemented in our population made possible an early diagnosis and treatment, trying to reduce the consequences. Diabetic retinopathy is widely known, however we present the case of a rare retinal condition, acute panedothelial retinal leakage. It typically affects patients with type 1 DM and poor glycemic control. Its diagnosis is important since a strict metabolic control is sufficient for its resolution, avoiding aggressive therapies.

Multimodal imaging in multiple evanescent white dot syndrome and new insights in pathogenesis.

PURPOSE: To report the multimodal imaging in multiple evanescent white dot syndrome (MEWDS) during the acute and convalescent stages in order to better understand the focus of the inflammatory process. METHODS: Retrospective cohort study of 4 patients with MEWDS. Each patient underwent: enhanced depth imaging-optical coherence tomography (EDI-OCT), fundus autofluorescence (FAF), fluorescein angiography (FA), indocyanine green angiography (ICGA) and en-face OCT and OCT angiography (OCT-A). Choroidal subfoveal thickness (CST) was measured manually. All patients were studied in the acute stage and convalescent stage after disappearance of OCT abnormalities and resolution of visual symptoms. RESULTS: Four MEWDS patients with a mean age of 23.5years were studied (range: 16-33years). Two patients were women. Initial mean visual acuity (VA) was 80.25 ETDRS. Final mean VA was 84.25 ETDRS. OCT imaging showed disruption of the ellipsoid zone and a slightly elevated RPE layer with overlying hyperreflective material, all of which corresponded to hyperautofluorescent FAF lesions. FA revealed multiple hyperautofluorescent lesions, correlated with hypocyanescent spots on the late ICGA. OCT-A showed normal superficial and deep retinal capillary plexus as well as choriocapillaris. The disease was self-limited in all the cases, with a mean time of 9weeks to resolution (range: 4-16). CONCLUSION: The pathophysiology of MEWDS is still debated. We believe that there is still not enough evidence to implicate the outer retina as the primary cause. For now, we suggest that this transient disease is the consequence of choriocapillaris hypoperfusion, but further studies are required to elucidate this hypothesis.

Capilaropatía edematosa aguda en un paciente con diabetes mellitus tipo 1 con mal control metabólico / Acute panendothelial retinal leakage in a patient with diabetes mellitus type 1 with a poor metabolic control

Las complicaciones oftalmológicas derivadas de la diabetes son una de las principales causas de ceguera en edad laboral a nivel mundial, por lo que es importante un manejo minucioso. Gracias a los planes de cribado implantados en nuestra población, es posible un diagnóstico y tratamiento precoz con el fin de disminuir las secuelas. La retinopatía diabética (RD) es una entidad conocida por todos nosotros, no obstante, presentamos el caso de una rara afectación retiniana, la capilaropatía edematosa aguda. Típicamente afecta a pacientes con diabetes mellitus tipo 1 (DM1) y mal control glucémico. Es importante su diagnóstico puesto que llevar un estricto control metabólico es suficiente para la resolución de la misma, evitando terapias agresivas (AU)

Ophthalmological diabetic complications are one of the main causes of blindness worldwide, so careful diagnostic and management is important. The screening plans implemented in our population made possible an early diagnosis and treatment, trying to reduce the consequences. Diabetic retinopathy is widely known, however we present the case of a rare retinal condition, acute panedothelial retinal leakage. It typically affects patients with diabetes mellitus (DM) type 1 and poor glycemic control. Its diagnosis is important since a strict metabolic control is sufficient for its resolution, avoiding aggressive therapies (AU)

Acute panendothelial retinal leakage in a patient with diabetes mellitus type 1 with a poor metabolic control. / Capilaropatía edematosa aguda en un paciente con diabetes mellitus tipo 1 con mal control metabólico.

Ophthalmological diabetic complications are one of the main causes of blindness worldwide, so careful diagnostic and management is important. The screening plans implemented in our population made possible an early diagnosis and treatment, trying to reduce the consequences. Diabetic retinopathy is widely known, however we present the case of a rare retinal condition, acute panedothelial retinal leakage. It typically affects patients with diabetes mellitus (DM) type 1 and poor glycemic control. Its diagnosis is important since a strict metabolic control is sufficient for its resolution, avoiding aggressive therapies.

Pseudoxanthoma elasticum in childhood in patient with ß-thalassaemia. / Pseudoxantoma elástico infantil en paciente con ß-talasemia.

Pseudoxanthoma elasticum (PXE) is a rare genetic disorder characterised by elastic tissue alterations and caused by mutations in a single gene, ABCC6, on chromosome 16p that includes manifestations that are predominantly cutaneous, ocular and cardiovascular. PXE-like lesions in association with ß-thalassemia have previously been reported in the literature in patients with ß-thalassaemia intermediate and major, being clinically indistinguishable from classic PXE. The case is presented of a 10-year-old boy with ß-thalassaemia minor and characteristic lesions of PXE. It is worth noting the benefit of multimodal imaging in the diagnosis and monitoring of the lesions.

Toxicidad macular precoz tras 2 meses de tratamiento con hidroxicloroquina / Early macular toxicity following 2 months of hydroxychloroquine therapy

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Nuevos hallazgos peripapilares en el síndrome de Alport: caso clínico / New peripapillary findings in Alport syndrome: A case report

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[Acute macular neuroretinopathy]. / Neurorretinopatía macular aguda.

CLINICAL CASE: A 22-year-old woman complained of symptoms consistent with the sudden onset of central scotomas in both eyes after a flu-like illness. The typical reddish brown macular lesions of acute macular neuroretinopathy were evident in the parafoveal area of both eyes. Visual field examination revealed central scotomas in both eyes that corresponded in location to the retinal injuries. DISCUSSION: Acute macular neuroretinopathy is a rare idiopathic posterior uveitis for which no effective treatment is available. Several pathogenic theories are postulated.

Neurorretinopatía macular aguda / Acute macular neuroretinopathy

Caso clínico: Mujer de 22 años que refería la aparición brusca de escotomas centrales en ambos ojos tras cuadro catarral de vías respiratorias altas. En el fondo del ojo (FO) presentaba unas manchas redondeadas parafoveales de color marrón rojizo. Los campos visuales presentaban unos escotomas centrales que se correspondían con la localización de las lesiones retinianas. Discusión: La neurorretinopatía macular aguda (NMA) es una uveítis posterior idiopática, poco frecuente, sin tratamiento efectivo y de la que existen diversas teorías sobre su patogenia

Clinical case: A 22-year-old woman complained of symptoms consistent with the sudden onset of central scotomas in both eyes after a flu-like illness. The typical reddish brown macular lesions of acute macular neuroretinopathy were evident in the parafoveal area of both eyes. Visual field examination revealed central scotomas in both eyes that corresponded in location to the retinal injuries. Discussion: Acute macular neuroretinopathy is a rare idiopathic posterior uveitis for which no effective treatment is available. Several pathogenic theories are postulated

[Recurrent uveitis and therapy with monoclonal antibody (daclizumab)]. / Uveítis recurrentes y tratamiento con anticuerpos monoclonales (Daclizumab).

PURPOSE: Assess the efficacy of monoclonal antibody (daclizumab) therapy in the treatment of patients with non-infectious uveitis who do not respond to other immunosuppressive agents. MATERIAL AND METHOD: Ten patients with non-infectious intermediate and posterior uveitis were treated with intravenous daclizumab (humanized anti-IL-2 receptor monoclonal antibody) monthly for two years. RESULTS: 70% of the patients have had some improvement in their inflammatory activity being maintained without recurrence. 10% of the patients improved their visual acuity in two lines and the macular edema decreased. 20% of the patients continued to have recurrences, but not worse than the year before. 40% of the patients gave up having the treatment with corticosteroids and 30% with minimum dose (5 mg). No secondary effects were noted during the treatment. CONCLUSIONS: Daclizumab is effective and safe for long-term use in the treatment of uveitis which cannot be kept under control by other treatments. In time, it allows the dose of corticosteroids to be decreased or even discontinued.