Clinical significance of ocular manifestations in granulomatosis with polyangiitis: association with sinonasal involvement and damage.

Ocular involvement is present in 50-60% of granulomatosis with polyangiitis (GPA) patients and can affect any part of the ocular globe. The present study describes ophthalmologic manifestations, association with systemic symptoms, disease activity and damage in GPA. A cross-sectional study was conducted including patients with GPA who underwent rheumatologic and ophthalmologic evaluation. Demographics, comorbidities, ophthalmologic symptoms, serologic markers, radiographic studies, disease activity and damage were assessed. Descriptive statistics, correlation, univariable logistic regression analyses, Student's t, Mann-Whitney U, Chi-square and Fisher's exact tests were performed. Fifty patients were included, 60% female, the median age was 56 years, disease duration 72.5 months. Nineteen (38%) patients had ocular manifestations at GPA diagnosis, scleritis being the most frequent; 27 (54%) patients presented ocular involvement during follow-up, repeated scleritis and dacryocystitis being the most common manifestations. Concomitant ophthalmic and sinonasal involvement was present in 12 (24%). Ocular and ENT damage occurred in 58% and 70%, respectively. Epiphora and blurred vision were the most frequent symptoms; scleromalacia and conjunctival hyperemia (27%) the most frequent clinical abnormalities. Ocular involvement at diagnosis was associated with concomitant ocular and sinonasal involvement at follow-up (OR 4.72, 95% CI 1.17-19.01, p = 0.01). Ocular involvement at follow-up was associated with age at GPA diagnosis (OR 0.94, 95% CI 0.90-0.99, p = 0.03), VDI (OR 1.29, 95% CI 1.03-1.61, p = 0.02), and ENT damage (OR 5.27, 95% CI 1.37-20.13, p = 0.01). In GPA, ocular involvement is frequent, therefore, non-ophthalmologist clinicians should be aware of this manifestation to reduce the risk of visual morbidity and organ damage.

Validación de la prueba de hielo en oftalmoparesia por miastenia grave / Validation of the ice pack test in ophthalmoparesis due to myasthenia gravis

Introducción. La miastenia grave es una enfermedad autoinmune de la unión neuromuscular que se presenta clínicamente como debilidad fluctuante de los músculos estriados, como los de la región ocular (miastenia ocular). Objetivo. Demostrar que la sensibilidad y la especificidad de la prueba de hielo son altas en el diagnóstico diferencial de la oftalmoparesia y ptosis palpebral por miastenia grave y miastenia ocular. Sujetos y métodos. Estudio observacional, analítico, no aleatorizado, de una muestra de 43 sujetos, 21 con miastenia grave y 22 controles. A todos los pacientes se les aplicó un guante con hielo sobre sus párpados superiores afectados durante dos minutos, después de los cuales se evaluó el grado de mejoría de la ptosis palpebral y la oftalmoparesia. Todos tenían estudio de estimulación nerviosa repetitiva. Resultados. Se analizaron 36 pacientes, 18 con miastenia grave u ocular y 18 controles. Todos presentaron ptosis palpebral y sólo 20 de ellos oftalmoparesia. La prueba de hielo para la oftalmoparesia mostró una sensibilidad del 83%, especificidad del 100%, valor predictivo positivo (VPP) del 100% y valor predictivo negativo (VPN) del 80% en el diagnóstico de la miastenia grave. Para la ptosis palpebral, se determinó una sensibilidad del 89%, especificidad del 100%, VPP del 100% y VPN del 90%. Para la estimulación nerviosa repetitiva se calculó una sensibilidad del 61%, especificidad del 83%, VPP del 79% y VPN del 68%. Conclusión. La prueba de hielo es sencilla, segura, económica, rápida y fiable para utilizarse de rutina en pacientes con sospecha de ptosis u oftalmoparesia por miastenia grave, ya que tiene una alta validez, seguridad y reproducibilidad como prueba diagnóstica (AU)

Introduction. Myasthenia gravis is an autoimmune disease of the neuromuscular junction that presents clinically asn fluctuating weakness of skeletal muscles, as of the ocular region (myasthenia ocular). Aim. To demonstrate that the sensitivity and specificity of the ice pack test are high, in the differential diagnosis of palpebral ptosis and ophthalmoparesis for myasthenia gravis and myasthenia ocular. Subjects and methods. Observational, analytical, and non-randomized study of 43 subjects, 21 with myasthenia gravis and 22 controls. All patients received a glove with ice on your upper eyelids affected for 2 minutes, after which we assessed the degree of improvement in palpebral ptosis and ophthalmoparesis. All patients had repetitive nerve stimulation study. Results. We analyzed 36 patients, 18 patients with myasthenia gravis or myasthenia ocular and 18 controls. All patients had palpebral ptosis but ophthalmoparesis only 20 of them. Ice pack test for ophthalmoparesis showed a sensitivity of 83%, specificity 100%, positive predictive value (PPV) of 100% and negative predictive value (NPV) of 80% in the diagnosis of myasthenia gravis. Ice pack test for palpebral ptosis was determined a sensitivity of 89%, specificity 100%, PPV of 100% and NPV of 90%. For repetitive nerve stimulation was calculated a sensitivity of 61%, specificity of 83%, PPV of 79% and NPV of 68%. Conclusion. Ice pack test, both palpebral ptosis and ophthalmoparesis is a simple test, safe, cheap, fast and reliable to be used routinely in patients suspected of palpebral ptosis and/or ophthalmoparesis due to myasthenia gravis, and which has a high validity, safety, and reproducibility as a diagnostic test (AU)

[Validation of the ice pack test in ophthalmoparesis due to myasthenia gravis]. / Validacion de la prueba de hielo en oftalmoparesia por miastenia grave.

INTRODUCTION: Myasthenia gravis is an autoimmune disease of the neuromuscular junction that presents clinically as fluctuating weakness of skeletal muscles, as of the ocular region (myasthenia ocular). AIM: To demonstrate that the sensitivity and specificity of the ice pack test are high, in the differential diagnosis of palpebral ptosis and ophthalmoparesis for myasthenia gravis and myasthenia ocular. SUBJECTS AND METHODS: Observational, analytical, and non-randomized study of 43 subjects, 21 with myasthenia gravis and 22 controls. All patients received a glove with ice on your upper eyelids affected for 2 minutes, after which we assessed the degree of improvement in palpebral ptosis and ophthalmoparesis. All patients had repetitive nerve stimulation study. RESULTS: We analyzed 36 patients, 18 patients with myasthenia gravis or myasthenia ocular and 18 controls. All patients had palpebral ptosis but ophthalmoparesis only 20 of them. Ice pack test for ophthalmoparesis showed a sensitivity of 83%, specificity 100%, positive predictive value (PPV) of 100% and negative predictive value (NPV) of 80% in the diagnosis of myasthenia gravis. Ice pack test for palpebral ptosis was determined a sensitivity of 89%, specificity 100%, PPV of 100% and NPV of 90%. For repetitive nerve stimulation was calculated a sensitivity of 61%, specificity of 83%, PPV of 79% and NPV of 68%. CONCLUSION: Ice pack test, both palpebral ptosis and ophthalmoparesis is a simple test, safe, cheap, fast and reliable to be used routinely in patients suspected of palpebral ptosis and/or ophthalmoparesis due to myasthenia gravis, and which has a high validity, safety, and reproducibility as a diagnostic test.

TITLE: Validacion de la prueba de hielo en oftalmoparesia por miastenia grave.Introduccion. La miastenia grave es una enfermedad autoinmune de la union neuromuscular que se presenta clinicamente como debilidad fluctuante de los musculos estriados, como los de la region ocular (miastenia ocular). Objetivo. Demostrar que la sensibilidad y la especificidad de la prueba de hielo son altas en el diagnostico diferencial de la oftalmoparesia y ptosis palpebral por miastenia grave y miastenia ocular. Sujetos y metodos. Estudio observacional, analitico, no aleatorizado, de una muestra de 43 sujetos, 21 con miastenia grave y 22 controles. A todos los pacientes se les aplico un guante con hielo sobre sus parpados superiores afectados durante dos minutos, despues de los cuales se evaluo el grado de mejoria de la ptosis palpebral y la oftalmoparesia. Todos tenian estudio de estimulacion nerviosa repetitiva. Resultados. Se analizaron 36 pacientes, 18 con miastenia grave u ocular y 18 controles. Todos presentaron ptosis palpebral y solo 20 de ellos oftalmoparesia. La prueba de hielo para la oftalmoparesia mostro una sensibilidad del 83%, especificidad del 100%, valor predictivo positivo (VPP) del 100% y valor predictivo negativo (VPN) del 80% en el diagnostico de la miastenia grave. Para la ptosis palpebral, se determino una sensibilidad del 89%, especificidad del 100%, VPP del 100% y VPN del 90%. Para la estimulacion nerviosa repetitiva se calculo una sensibilidad del 61%, especificidad del 83%, VPP del 79% y VPN del 68%. Conclusion. La prueba de hielo es sencilla, segura, economica, rapida y fiable para utilizarse de rutina en pacientes con sospecha de ptosis u oftalmoparesia por miastenia grave, ya que tiene una alta validez, seguridad y reproducibilidad como prueba diagnostica.