IgG4-related Disease and the Liver

• Autores: Jonathan H. Chen, Vikram Deshpande
• Localización: Gastroenterology clinics of North America, ISSN 0889-8553, Vol. 46, Nº. 2, 2017 (Ejemplar dedicado a: Liver Pathology), págs. 195-216
• Idioma: inglés
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• Resumen

  • Pathologists are likely to encounter IgG4-related disease in several organ systems. This article focuses on helping pathologists diagnose IgG4-related disease in the hepatobiliary system. Missing the diagnosis can result in unnecessary organ damage and/or unnecessary surgical and cancer therapy. In the liver, tumefactive lesion(s) involving the bile ducts with storiform fibrosis and an IgG4-enriched lymphoplasmacytic infiltrate are highly concerning for IgG4-related disease. The recent identification of oligoclonal populations of T cells and B cells in IgG4-related disease may lead to molecular tests, new therapeutics, and a greater mechanistic understanding of the disease.