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Introducción: la enfermedad relacionada con IgG4 (ER-IgG4) es una entidad reciente, caracterizada por infiltración de plasmocitos IgG4+ y esclerosis de patrón estoriforme mediada por linfocitos CD4+ y T-foliculares ayudadores (Tfh). La mayoría de los pacientes presentan adenopatías y pérdida de peso. Entre los sitios más afectados se encuentran páncreas, glándulas salivales y lacrimales, aorta y retroperitoneo, siendo raro el compromiso gástrico. El diagnóstico se establece en el estudio histopatológico del órgano comprometido, la clínica y la serología. Los glucocorticoides constituyen la primera línea de manejo. Presentación del caso: paciente masculino de 38 años con síntomas sugestivos de sangrado digestivo superior, la endoscopia de vías digestivas altas reveló una ulceración en el cuerpo del estómago, sospechosa de una lesión subepitelial gástrica ulcerada, de posible origen del estroma gástrico (GIST), por lo que se realizó gastrectomía subtotal por vía laparoscópica. La histología fue compatible con un tumor ulcerado fibroesclerosante relacionado con IgG4. Se inició tratamiento con glucocorticoides con respuesta favorable. Conclusión: en esta revisión se analizarán los casos de compromiso gástrico encontrados en la literatura para describir las características más importantes de esta entidad.
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