Periarteritis/periaortitis y fibrosis retroperitoneal en enfermedad relacionada por IgG4

Luisina Zunino, Sergio Paira

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Resumen

La enfermedad relacionada a IGg4 es una afección fibroinflamatoria sistémica que cobra cada vez más relevancia. Se caracteriza por una triada clásica en el análisis histopatológico: Infiltrado Linfoplasmocitario, Fibrosis Estoriforme y Flebitis obliterante. El compromiso cardiovascular no queda fuera de su espectro pudiendo afectar a las arterias coronarias, las válvulas cardíacas, el miocardio, el pericardio, la aorta y la vasculatura periférica, confundiéndose con otras entidades reumatológicas que cursan con este compromiso. Resulta entonces fundamental reconocer las características clínicas e imagenológicas que la diferencian de las demás etiologías inflamatorias para su sospecha clínica y un óptimo accionar evitando complicaciones potencialmente fatales.

La existencia de nuevos criterios órgano específicos pueden facilitar el diagnóstico de dicho compromiso. La afección vascular asociado con IgG4 puede causar complicaciones potencialmente mortales, como ruptura de aneurismática y estenosis arterial; sin embargo, los detalles de las características clínicas, el pronóstico y el tratamiento óptimo siguen sin ser claros debido a la rareza de esta afección.

Palabras clave

enfermedad relacionada a IgG4; IgG4; aortitis; periaortitis

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