Lower Incidence of Biliary Carcinoma in Patients With Primary Sclerosing Cholangitis and High Serum Levels of Immunoglobulin E

2012 ◽  
Vol 10 (1) ◽  
pp. 79-83 ◽  
Author(s):  
Kenji Hirano ◽  
Minoru Tada ◽  
Suguru Mizuno ◽  
Hiroyuki Isayama ◽  
Naminatsu Takahara ◽  
...  
Keyword(s):  
Primary Sclerosing Cholangitis ◽  
Lower Incidence ◽  
Sclerosing Cholangitis ◽  
Immunoglobulin E ◽  
Serum Levels ◽  
High Serum ◽  
Biliary Carcinoma

scholarly journals Association Between HLA Haplotypes and Increased Serum Levels of IgG4 in Patients With Primary Sclerosing Cholangitis

2015 ◽  
Vol 148 (5) ◽  
pp. 924-927.e2 ◽  
Author(s):  
Natalie L. Berntsen ◽  
Olav Klingenberg ◽  
Brian D. Juran ◽  
Maria Benito de Valle ◽  
Björn Lindkvist ◽  
...  
Keyword(s):  
Primary Sclerosing Cholangitis ◽  
Sclerosing Cholangitis ◽  
Serum Levels ◽  
Hla Haplotypes

scholarly journals Administration of Human MSC-Derived Extracellular Vesicles for the Treatment of Primary Sclerosing Cholangitis: Preclinical Data in MDR2 Knockout Mice

2020 ◽  
Vol 21 (22) ◽  
pp. 8874
Author(s):  
Roberta Angioni ◽  
Bianca Calì ◽  
Vasanthy Vigneswara ◽  
Marika Crescenzi ◽  
Ana Merino ◽  
...  
Keyword(s):  
Primary Sclerosing Cholangitis ◽  
Extracellular Vesicles ◽  
Sclerosing Cholangitis ◽  
Serum Levels ◽  
Portal Triad ◽  
Immune Mediated ◽  
Nfkb Activation ◽  
Effective Therapeutic Strategy ◽  
Progressive Liver Disease ◽  
Clinical Experiments

Primary Sclerosing Cholangitis (PSC) is a progressive liver disease for which there is no effective medical therapy. PSC belongs to the family of immune-mediated biliary disorders and it is characterized by persistent biliary inflammation and fibrosis. Here, we explored the possibility of using extracellular vesicles (EVs) derived from human, bone marrow mesenchymal stromal cells (MSCs) to target liver inflammation and reduce fibrosis in a mouse model of PSC. Five-week-old male FVB.129P2-Abcb4tm1Bor mice were intraperitoneally injected with either 100 µL of EVs (± 9.1 × 109 particles/mL) or PBS, once a week, for three consecutive weeks. One week after the last injection, mice were sacrificed and liver and blood collected for flow cytometry analysis and transaminase quantification. In FVB.129P2-Abcb4tm1Bor mice, EV administration resulted in reduced serum levels of alkaline phosphatase (ALP), bile acid (BA), and alanine aminotransferase (ALT), as well as in decreased liver fibrosis. Mechanistically, we observed that EVs reduce liver accumulation of both granulocytes and T cells and dampen VCAM-1 expression. Further analysis revealed that the therapeutic effect of EVs is accompanied by the inhibition of NFkB activation in proximity of the portal triad. Our pre-clinical experiments suggest that EVs isolated from MSCs may represent an effective therapeutic strategy to treat patients suffering from PSC.

scholarly journals Advances in IgG4-related Hepatobiliary Disease

2020 ◽  
Vol 25 (2) ◽  
pp. 100-106
Author(s):  
Sung-Hoon Moon
Keyword(s):  
Primary Sclerosing Cholangitis ◽  
Sclerosing Cholangitis ◽  
Quantitative Polymerase Chain Reaction ◽  
High Serum ◽  
Hepatobiliary Disease ◽  
Glucocorticoid Therapy ◽  
Glucocorticoid Treatment ◽  
Immunoglobulin G4 ◽  
Serum Igg4 ◽  
Polymerase Chain

The emergence of the new disease entity of steroid responsive immunoglobulin G4 (IgG4)-related hepatobiliary disease has generated considerable interest among hepatobiliary society. IgG4-related hepatobiliary disease refers to mainly IgG4-related sclerosing cholangitis (IgG4-SC), and includes a small number of IgG4-related hepatopathy. As IgG4-SC responds well to glucocorticoid therapy, IgG4-SC should be differentiated from cholangiocarcinoma and primary sclerosing cholangitis. Timely diagnosis of IgG4-SC can lead clinicians to prescribe adequate glucocorticoid treatment that can reverse bile duct strictures and cholestatic liver function. Differentiation of IgG4-SC from primary sclerosing cholangitis is sometimes challenging because serum IgG4 and tissue IgG4 have demonstrated low positive predictive value in this setting. Recent research suggested that blood IgG4/IgG RNA ratio by quantitative polymerase chain reaction can be used for differentiation. Although most patients with IgG4-SC/autoimmune pancreatitis respond to glucocorticoid therapy, they frequently experience relapse of disease. The suggested relapse factors included very high serum IgG4, diffuse enlargement of the pancreas, proximal IgG4-SC, and multi-organ involvement. This review discusses the recent advances in the pathogenesis, diagnosis, management, disease relapse, and monitoring disease activity of IgG4-SC.

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