Type 1 autoimmune pancreatitis

Gustavo Mesquita De Oliveira,Lia De Freitas Araújo Alves,Clóvis Rêgo Coelho,Ana Luiza Viana Pequeno,Rodrigo Vieira Costa Lima,Paloma Lucena Cabral

doi:10.11606/issn.2176-7262.v53i1p81-87

Gustavo Mesquita De Oliveira, Lia De Freitas Araújo Alves + Show 4 more

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https://doi.org/10.11606/issn.2176-7262.v53i1p81-87

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Abstract

Type 1 autoimmune pancreatitis is a cause of chronic pancreatitis related to the systemic disease known as IgG4-related Sclerosing Disease. Case report: We report the case of a 64-year-old male patient who presented recurrent epigastric pain radiating to the back, associated with jaundice, xerostomia, nausea, and vomiting, since 2014, diagnosed two years later with an unresectable pancreatic adenocarcinoma. The diagnosis was questioned after a few follow-up months without clinical deterioration when it was suggested the possibility of type 1 autoimmune pancreatitis in its pseudotumoral form. The patient was then treated with glucocorticoids, obtaining significant clinical improvement. After two years of follow-up, he returned asymptomatic with images suggestive of sclerosing cholangitis and a large liver abscess. Importance of the issue: The present case denotes the difficulty found in this diagnosis due to clinical and radiological resemblances with pancreatic adenocarcinoma. Besides that, it presents a seldom described disease complication, the liver abscess.

Highlights

IgG4-related disease (IgG4-RD), known as IgG4-related Sclerosing Disease, is a recently defined entity characterized by serum elevated levels of IgG4, lymphoplasmacytic infiltrate and an immune-mediated fibroinflammatory process in the organs affected
This is a systemic disease associated with pancreatic impairment recognized as Type 1 Autoimmune Pancreatitis (AIP), a rare form of chronic pancreatitis
This article reports a case of a patient with AIP who recurred with a liver abscess associated with sclerosing cholangitis

Summary

INTRODUCTION

IgG4-related disease (IgG4-RD), known as IgG4-related Sclerosing Disease, is a recently defined entity characterized by serum elevated levels of IgG4, lymphoplasmacytic infiltrate and an immune-mediated fibroinflammatory process in the organs affected. In November 2016, the patient presented for the first time high fasting glucose levels with the hypothesis of Endocrine Pancreatic Insufficiency worsened by glucocorticoid use He was admitted in December of the same year due to difficulties in glycemic control. After discharge from the hospital, the patient maintained an outpatient follow-up every 4 months, and the corticosteroid weaning was performed progressively He remained asymptomatic with periodic follow-up and prednisone was discontinued after about a year and a half of the beginning of corticotherapy. There were described areas of discontinuous narrowing of the intrahepatic bile ducts suggestive of sclerosing cholangitis During hospitalization, he denied fever, weight loss, jaundice, loss of appetite or abdominal pain. At the time of presenting this case report, he remained asymptomatic with no hepatic collection observed in control ultrasonography and is using azathioprine as maintenance therapy, with good tolerance and no drug-related side effects

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CONCLUSION