Sclerosing cholangitis associated with IgG4 disease. Case Report

Introduction and Objectives

This study aimed to present the case of a patient with sclerosing cholangitis and autoimmune pancreatitis associated with IgG4 disease.

Materials and Methods

A 44-year-old male with a history of allergic rhinitis, diabetes, and dyslipidemia. His clinical picture began with diffuse abdominal pain and jaundice; on physical examination, jaundice, a painful abdomen in the right upper quadrant and hepatomegaly. Liver biochemistry with transaminasemia and cholestasis. In auxiliary studies US with bile duct dilatation of 12.4 mm, ColangioMR was performed, reporting concentric wall thickening in T2 of the intra and extrahepatic bile duct that condition focal stenoses of the right hepatic duct and intrahepatic bile duct approximately 16 mm from the ampulla, conditioning segmental dilatations of the common hepatic and extrapancreatic bile duct up to 8.9mm. According to these findings, IgG4 serum levels were requested, reporting 1200mg/dl. Steroid treatment was started, presenting a favorable response.

Discussion

IG-G4-related disease is an autoimmune relapsing chronic multiorgan fibroinflammatory syndrome. Its maximum incidence is in Japan, with 336 to 1300 patients diagnosed/year; the estimated prevalence is 62/million subjects between 50 and 70 years of age. The main and most commonly affected organs are the pancreas, bile duct, salivary and lacrimal glands, retroperitoneum and lymph nodes.

Conclusions

The relevance as Gastroenterologists recognize the disease associated with IgG4 because of the multi-organ involvement as part of the approach to a patient with jaundice syndrome; despite the low prevalence reported in our country, knowing this entity will make its timely treatment and subsequent recognition easier in other patients.

Funding

The resources used in this study were from the hospital without any additional financing

Declaration of interest

The authors declare no potential conflicts of interest.