CASE PRESENTATION: We describe a case of a @entity1 who presents with @entity1087 , elevated cholestatic liver enzymes, an extreme @entity353 and a midcholedochal stricture very suspect for a @entity201 . In the conviction of malignancy, although the absence of anatomopathological prove, the @entity1 underwent a choledochal resection. The anatomopathological specimen revealed no malignancy. In the year following resection, the @entity1 keeps presenting with bile duct strictures and further @entity353 . Ultimately the diagnosis of Ig G4-related @entity255 is withheld. Therapy with corticosteroids is initiated with a spectacular clinical, biochemical and radiographical result. DISCUSSION: IgG4-related @entity255 is the biliary presentation of IgG4-related disease, a recently discovered entity of fibroinflammatory masses which can affect virtually every organ in the body. It is characterized by a dense lymphoplasmacytic infiltrate, storiform @entity82 , obliterative @entity3527 and a presence of > 30 IgG4-positive plasma cells per high power field. Main differential diagnosis contains @entity201 and @entity991 . Corticoids are cornerstone of therapy, with @entity500 frequently used as a maintenance in case of relapse. CONCLUSIONS: With this case we want to draw the attention to a rather uncommon cause of @entity253 , easily mistaken for a @entity201 .

IgG4-related XXXX : Case report and literature review.

@entity1 :: ('9606', 'Species') :: ['patient'] @entity82 :: ('MESH:D005355', 'Disease') :: ['fibrosis'] @entity1087 :: ('MESH:D007565', 'Disease') :: ['jaundice'] @entity353 :: ('MESH:D015431', 'Disease') :: ['weight loss'] @entity500 :: ('MESH:D001379', 'Chemical') :: ['azathioprine'] @entity991 :: ('MESH:D015209', 'Disease') :: ['primary sclerosing cholangitis'] @entity201 :: ('MESH:D018281', 'Disease') :: ['cholangiocarcinoma'] @entity253 :: ('MESH:D001660', 'Disease') :: ['biliary obstruction'] @entity3527 :: ('MESH:D010689', 'Disease') :: ['phlebitis'] @entity255 :: ('MESH:D002761', 'Disease') :: ['cholangitis']
@entity255 :: (MESH:D002761,Disease) :: ['cholangitis']
