Abstract

A 59-year old women developed spontaneous jaundice without fever and after a progressive loss of 10 kg in 6 months. Iconography showed an enlarged gallbladder with gallstones and thickening walls responsible for an intrahepatic bile duct dilatation with infiltration of the hepatic pedicle and a portal stenosis (Fig. 1). Jaundice spontaneously resolved but cholestasis remained. Surgical and radiological guided biopsy were performed twice without contributive histology. We decided to proceed to a right portal vein embolization in the aim to proceed to an extended right hepatectomy. Fortunately, definitive histology of the last biopsy revealed inflammatory condition with a high IgG4-plasmocytes rate although the patient's serum concentration of IgG4 was moderate (1.39 g/L, normal rate: 0.86–1.35 g/L) and initially considered as not significant. A complete check up was performed and concluded that it was the unique location of an IgG4-related disease [ [1] Lanzillotta M. Mancuso G. Della-Torre E. Advances in the diagnosis and management of IgG4 related disease. BMJ. 2020; 369: m1067https://doi.org/10.1136/bmj.m1067 Crossref PubMed Scopus (97) Google Scholar ]. Corticotherapy was introduced.

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