Enfermedad relacionada con IgG4

  1. Pretel Ruiz, P. 1
  2. Pérez Gómez, A.M. 1
  3. Rabadán Rubio, E. 1
  4. Álvarez-Mon Soto, M. 1
  1. 1 Servicio de Enfermedades del Sistema Inmune-Reumatología, Hospital Príncipe de Asturias, Alcalá de Henares, Madrid, España
Revista:
Medicine: Programa de Formación Médica Continuada Acreditado

ISSN: 0304-5412

Año de publicación: 2021

Título del ejemplar: Enfermedades del sistema inmune (V)

Serie: 13

Número: 32

Páginas: 1809-1823

Tipo: Artículo

DOI: 10.1016/J.MED.2021.04.011 DIALNET GOOGLE SCHOLAR

Otras publicaciones en: Medicine: Programa de Formación Médica Continuada Acreditado

Resumen

La enfermedad relacionada con la inmunoglobulina G4 (ER-IgG4) es una condición fibroinflamatoria que puede involucrar a todos los sistemas del organismo. Debido a la descripción relativamente reciente de esta enfermedad, en las primeras décadas del año 2000, por el momento, su etiología y fisiopatología no están suficientemente detalladas. Cada vez son más las pruebas de que la patogénesis de la enfermedad tiene una base autoinmune, con participación de las células B de memoria, las células T helper foliculares (Thf), los linfocitos T CD4 y los macrófagos. Inicialmente, la elevación de los niveles de la fracción 4 de la IgG (IgG4) en el suero de estos pacientes hizo pensar que podrían tener un importante valor en la etiopatogenia y el diagnóstico. Sin embargo, con posterioridad, se ha observado que tan solo el 60% de los pacientes, aproximadamente, presenta niveles de IgG4 elevados en suero. Aunque puede afectar prácticamente a cualquier órgano, se han ido definiendo predilecciones, principalmente las glándulas salivales, órbitas y glándulas lagrimales, páncreas y el árbol biliar, pulmones, riñones, aorta y el retroperitoneo, meninges y glándula tiroides. Todos los órganos afectados por la ER-IgG4 comparten unas características histopatológicas: infiltrado linfoplasmocitario con abundantes células plasmáticas IgG4 positivas, fibrosis con un patrón estoriforme, flebitis obliterante y presencia de eosinófilos. Realizar un diagnóstico es complejo, ya que requiere una combinación de examen clínico, analítico, radiológico y anatomopatológico. Esto, además, requiere la exclusión numerosos diagnósticos diferenciales.

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Fuente de los datos: Dialnet