58-year old woman who consulted for pruritus and liver panel alterations: AST 153 U/l, ALT 266 U/l, GGT 979 U/l, alkaline phosphatase 807 U/l with Ca 19.9 15 U/mL.
The magnetic resonance suggested malignant neoformation (Fig. 1). The endoscopic ultrasound-guided FNA revealed cellular atypia. A cephalic pancreatectomy was performed without metastasis or vascular invasion being observed. The rest of the pancreatic parenchyma was atrophic and fibrous. The histopathological study was consistent with IgG4-related type 1 autoimmune pancreatitis (Fig. 2).
Type 1 autoimmune pancreatitis is the pancreatic manifestation of IgG4-related disease.1 The histopathology2 is what was found in our case. This process involves other organs3; the most common is association with sclerosing cholangitis (70% cases).1
The patient was followed up and 4 years later she presented with cholestasis GGT 290 U/l and alkaline phosphatase 130 U/l without cytolysis. An increase in IgG of 1850 mg/dl (normal: 600–1600 mg/dl) and ANA + 1/160 were detected in the lab tests. The endoscopic ultrasound did not show any biliary alterations. A liver biopsy was performed and was consistent with cholangitis attributed to IgG4, although in this case the staining for IgG4 plasma cells was not positive (Fig. 3).
Corticosteroids associated with azathioprine were prescribed, obtaining a good response.
Please cite this article as: Ruiz Rebollo ML, Álvarez-Quiñones-Sanz M, Fuertes Alija JJ, Izquierdo Santervás S. Enfermedad IgG4-mediada simulando neoformación de páncreas. Gastroenterol Hepatol. 2021;44:660–661.
