Guidelines on the diagnosis and management of primary sclerosing cholangitis (2021) / 中华肝脏病杂志

In 2015, the first Chinese consensus on the diagnosis and management of primary sclerosing cholangitis was issued. In the past years, more data have emerged from the literature. Herein, the Autoimmune Liver Disease Group of the Chinese Society of Hepatology organized an expert group to review the evidence and updated the recommendations to formulate the guidelines. There are 21 recommendations on PSC clinical practice. To facilitate the differentiation between PSC and IgG4-related sclerosing cholangitis, 10 recommendations on IgG4-SC are also attached. These guidelines aim to provide a working reference for the management of PSC and IgG4-SC.

Challenges to Diagnosis and Management of Autoimmune Liver Diseases / 胃肠病学

Autoimmune liver diseases (AILDs),including mainly autoimmune hepatitis (AIH),primary biliary cholangitis (PBC),primary sclerosing cholangitis (PSC),IgG4-related sclerosing cholangitis (IgG4-SC),and overlap syndromes,are characterized by circulating autoantibodies,inflammatory liver histology,and increased level of serum immunoglobulins. Early diagnosis and management can significantly improve the prognosis of patients and their quality of life. This editorial focused on the research progress and difficulties encountered in studies on AILDs in China.

Cholangiocarcinoma Masquerading as IgG4-related Sclerosing Cholangitis / 대한췌담도학회지

IgG4-related sclerosing cholangitis (IgG4-SC) represents a rare but clinically challenging differential diagnosis in patients with biliary strictures which can be mistaken for cholangiocarcinoma. We present a case of a 73-year-old male presented with abdominal discomfort and weight loss. Biliary images showed long-segment luminal narrowing of extrahepatic bile duct associated with prominent enhanced wall thickening, but luminal patency was preserved. Pancreatic images revealed segmental irregular narrowing of main pancreatic duct without upstream duct dilatation. His liver function tests and CA19-9 level were normal. Putting all findings together, IgG4-SC associated with autoimmune pancreatitis was strongly suspected. However, endobiliary biopsy of extrahepatic bile duct revealed adenocarcinoma which was not resectable due to celiac axis involvement. Because there is an overlap in biliary imaging findings between IgG4-SC and cholangiocarcinoma, biopsy is essential for adequate differential diagnosis. We present a case of cholangiocarcinoma masquerading as IgG4-SC based on clinical and imaging findings.

Comparing the clinical characteristics of IgG4-related sclerosing cholangitis with primary sclerosing cholangitis / 中华肝胆外科杂志

Objective To analyze and compare the clinical characteristics of primary sclerosing cholangitis (PSC) with IgG4-related sclerosing cholangitis (IgG4-SC).Methods The clinical data of 32 PSC patients and 72 IgG4-SC patients who were hospitalized in Peking Union Medical College Hospital (PUMCH) from January 2004 to December 2014 were retrospectively analyzed.Results Of the 32 PSC patients,there were 16 male and 16 female.Of the 72 IgG4-SC patients,there were 61 male and 11 female,(ratio =5.5∶ 1).The average ages were 44.9 (11 ～ 77) and 59.8 (28 ～ 83) years,respectively (P ＜0.05).The most common symptoms of PSC and IgG4-SC were abdominal pain and jaundice,and the incidences of abdominal pain and jaundice were 50.0％ and 68.1％,78.1％ and 81.9％,respectively.The serum IgG4 level of the IgG4-SC patients was significantly higher than the PSC patients (P ＜ 0.05).The total protein in serum of the IgG4-SC patients was higher than the PSC patients (P ＜ 0.05).The rate of bile duct wall thickening as detected on endoscopic ultrasonography (EUS) was higher than by abdominal ultrasound and abdominal CT,which were 91.2％,11.5％ and 33.3％,respectively (P ＜0.05).12 PSC patients were followed up for over 2 years,including 2 patients who underwent liver transplantation after failure of conservative treatment,5 patients who died from hepatic failure and infection,and 3 with stable condition.43 IgG4-SC patients were followed up for over 2 years,including 16 patients with relapse.The recurrence rate was 37.2％ (16/43).The more the extrabiliary organs or bile duct segments were involved,the higher was the recurrence rate.Conclusions Both PSC and IgG4-SC are cholestatic diseases,and they have many similarities in clinical and imaging manifestations.However,they still have unique features.IgG4-SC is sensitive to glucocorticoids therapy and has good prognosis.Thus,it is important to differentiate PSC from IgG4-SC.

Recent Advances in the Concept and Pathogenesis of IgG4-Related Disease in the Hepato-Bilio-Pancreatic System

Recent studies have proposed nomenclatures of type 1 autoimmune pancreatitis (AIP) (IgG4-related pancreatitis), IgG4-related sclerosing cholangitis (IgG4-SC), IgG4-related cholecystitis, and IgG4-related hepatopathy as IgG4-related disease (IgG4-RD) in the hepato-bilio-pancreatic system. In IgG4-related hepatopathy, a novel concept of IgG4-related autoimmune hepatitis (AIH) with the same histopathological features as AIH has been proposed. Among organs involved in IgG4-RD, associations with pancreatic and biliary lesions are most frequently observed, supporting the novel concept of "biliary diseases with pancreatic counterparts." Targets of type 1 AIP and IgG4-SC may be periductal glands around the bile and pancreatic ducts. Based on genetic backgrounds, innate and acquired immunity, Th2-dominant immune status, regulatory T (Treg) or B cells, and complement activation via a classical pathway may be involved in the development of IgG4-RD. Although the role of IgG4 remains unclear in IgG4-RD, IgG4-production is upregulated by interleukin 10 from Treg cells and by B cell activating factor from monocytes/basophils with stimulation of toll-like receptors/nucleotide-binding oligomerization domain-like receptors. Based on these findings, we have proposed a hypothesis for the development of IgG4-RD in the hepato-bilio-pancreatic system. Further studies are necessary to clarify the pathogenic mechanism of IgG4-RD.

IgG4-related Sclerosing Cholangitis Presented as a Mass in the Liver / 대한췌담도학회지

Autoimmune pancreatitis or IgG4-related sclerosing cholangitis often involves the liver. Most common lesion involving the liver is shown as mass or masses often referred as inflammatory pseudotumor. Inflammatory pseudotumor usually needs to be discriminated with malignancy. Here we report a case of IgG4-related sclerosing cholangitis with liver involvement presented as a mass. It was proven by biopsy and did not show any evidence of autoimmune pancreatitis. The mass infiltrated around the portal tract and portal vein thrombosis was also present.

Usefulness of Intraductal Ultrasonography in the Diagnosis of Cholangiocarcinoma and IgG4-Related Sclerosing Cholangitis

The technique of intraductal ultrasonography (IDUS) of the bile duct with a thin-caliber probe and a ropeway system has provided excellent images of the bile duct and periductal structures and is an easy transpapillary approach. In addition, once the guide wire is inserted into the bile duct, IDUS and transpapillary biopsy after endoscopic retrograde cholangiopancreatography can be performed in a single session. Here, we review the usefulness of IDUS in the diagnosis of cholangiocarcinoma and IgG4-related sclerosing cholangitis.