Differential Diagnosis from Primary Sclerosing Cholangitis

Abstract

Primary sclerosing cholangitis (PSC), a chronic progressive disease of unknown etiology, is characterized by fibrosis and strictures involving the intra- and extrahepatic bile ducts [1, 2]. PSC is a distinct entity from IgG4-related sclerosing cholangitis (IgG4-SC), but some IgG4-SC masquerades as PSC by way of similar manifestations, such as frequent stenosis of both intra- and extrahepatic bile ducts, bile ductal wall thickening, male predominance, cholestatic liver dysfunction, and initial mild symptoms [3–7]. The differential diagnosis between PSC and IgG4-SC is clinically important because their treatment modalities and prognosis are very different [2, 7, 8]. Timely diagnosis of IgG4-SC can lead clinicians to prescribe adequate corticosteroid therapy that can reverse bile duct strictures/wall thickening and cholestatic liver dysfunction and could potentially prevent future advanced liver disease [2]. A proper diagnosis of PSC, in turn, is crucial for optimizing the surveillance of the disease progression to hepatic decompensation and the need for liver transplantation.