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Características generales de 29 pacientes con vasculitis de pequeños vasos / General characteristics of 29 patients with small vessel vasculitis
Di Benedetto, Nicolás; López Mujica, María Ximena; Fernández, Martín E.; Tourón, María; Muñoz, Sebastian A.; Allievi, Alberto.
  • Di Benedetto, Nicolás; Hospital Fernández. Servicio de Clínica Médica. Buenos Aires. AR
  • López Mujica, María Ximena; Hospital Fernández. Servicio de Clínica Médica. Buenos Aires. AR
  • Fernández, Martín E.; Hospital Fernández. Servicio de Clínica Médica. Buenos Aires. AR
  • Tourón, María; Hospital Fernández. Servicio de Clínica Médica. Buenos Aires. AR
  • Muñoz, Sebastian A.; Hospital Fernández. Servicio de Clínica Médica. Buenos Aires. AR
  • Allievi, Alberto; Hospital Fernández. Servicio de Clínica Médica. Buenos Aires. AR
Medicina (B.Aires) ; 70(2): 127-132, Apr. 2010. tab
Article in Spanish | LILACS | ID: lil-633731
RESUMEN
El objetivo de esta serie fue describir las características generales y manifestaciones clínicas de pacientes con vasculitis de pequeños vasos que concurrieron al servicio de autoinmunidad de un hospital de la comunidad y compararlas con la literatura. Se revisaron de manera retrospectiva las historias clínicas archivadas bajo el rótulo de vasculitis en un período de 16 años. Se seleccionaron aquellos pacientes que cumplieran criterios diagnósticos de vasculitis de pequeños vasos. Se extrajeron los datos de interés y se constató la supervivencia mediante contacto telefónico. Posteriormente se realizó una búsqueda bibliográfica y se compararon los resultados. Se incluyeron 13 pacientes con granulomatosis de Wegener, 6 con síndrome de Churg Strauss y 10 con poliangeítis microscópica. El 55% (16) fueron menores de 55 años al diagnóstico y hubo predominio del sexo femenino (2.61). La demora diagnóstica fue mayor a un año en el 46% de los casos. Los sistemas más frecuentemente afectados fueron respiratorio y otorrinolaringológico. El 79% presentó anticuerpos anti-citoplasma de neutrófilos (ANCA) positivos. La mortalidad fue del 24% (7/29). Al comparar las características de los pacientes incluidos en esta serie con lo descripto en la literatura, se hallaron diferencias en cuanto a la forma de presentación, sistemas comprometidos y porcentaje de pacientes ANCA positivos; además se observó que el resultado negativo de este marcador parece asociarse con mayor demora diagnóstica y en consecuencia peor pronóstico, lo que remarca su importancia como herramienta adicional no invasiva al momento del diagnóstico.
ABSTRACT
The objective of this series was to describe the general characteristics and clinical manifestations of patients with small vessel vasculitis who were assisted in the autoimmunity department of a community public hospital and to compare the results with the literature. Clinical records under the label of vasculitis in a period of 16 years were reviewed in a retrospective way. All patients selected fulfilled diagnostic criteria of small vessel vasculitis. The data were extracted and the analysis of survival was completed by phone. Later a bibliographical search was carried out and the results were compared. Thirteen patients with Wegener's granulomatosis, 6 with Churg-Strauss syndrome and 10 with microscopic polyangiitis were included. Fifty five percent (16) were under 55 years old when diagnosis was made and male/female ratio was 2.6 to 1. The diagnostic delay was over a year in 46% of the cases. Respiratory and ear-nose-throat were the most frequently affected systems. Anti-neutrophil cytoplasmic antibodies were present in 79% of patients. Overall mortality was 24% (7/29). There were several differences between the results of our series and the literature the presentation form, affected systems and percentage of patients with anti-neutrophil cytoplasmic antibodies. Greater diagnostic delay and worse prognosis were observed in anti-neutrophil cytoplasmic antibody negative patients. Special attention should be given to these antibodies since they constitute a significant tool at the time of diagnosis.
Subject(s)

Full text: Available Index: LILACS (Americas) Main subject: Churg-Strauss Syndrome / Granulomatosis with Polyangiitis / Antibodies, Antineutrophil Cytoplasmic / Microscopic Polyangiitis Type of study: Observational study / Prognostic study Limits: Adult / Aged / Female / Humans / Male Language: Spanish Journal: Medicina (B.Aires) Journal subject: Medicine Year: 2010 Type: Article Affiliation country: Argentina Institution/Affiliation country: Hospital Fernández/AR

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Full text: Available Index: LILACS (Americas) Main subject: Churg-Strauss Syndrome / Granulomatosis with Polyangiitis / Antibodies, Antineutrophil Cytoplasmic / Microscopic Polyangiitis Type of study: Observational study / Prognostic study Limits: Adult / Aged / Female / Humans / Male Language: Spanish Journal: Medicina (B.Aires) Journal subject: Medicine Year: 2010 Type: Article Affiliation country: Argentina Institution/Affiliation country: Hospital Fernández/AR