Clinical Utility of Personalized Serum IgG Subclass Ratios for the Differentiation of IgG4-Related Sclerosing Cholangitis (IgG4-SC) from Primary Sclerosing Cholangitis (PSC) and Cholangiocarcinoma (CCA)

Park, Jae Keun; Kim, Dongwuk; Lee, Jeong Min; Lee, Kwang Hyuck; Lee, Kyu Taek; Park, Joo Kyung; Lee, Jong Kyun

doi:10.3390/jpm12060855

Cited by 3 publications

(4 citation statements)

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“…Previously published studies on PSC and high IgG4 levels have been underpowered to assess the risk of malignancy in this specific cohort of patients [ 2 ]. Recent findings indicate that measurement of serum IgG4/IgG1 or IgG4/(IgG1+IgG3) levels may help to differentiate IgG4-related cholangitis from PSC [ 18 ]. In the same study, IgG4 alone was shown to be the most accurate serologic marker for the differentiation of IgG4-related cholangitis from cholangiocarcinoma [ 18 ].…”

Section: Discussionmentioning

confidence: 99%

“…Recent findings indicate that measurement of serum IgG4/IgG1 or IgG4/(IgG1+IgG3) levels may help to differentiate IgG4-related cholangitis from PSC [ 18 ]. In the same study, IgG4 alone was shown to be the most accurate serologic marker for the differentiation of IgG4-related cholangitis from cholangiocarcinoma [ 18 ]. IgG levels in our study were not associated with the development of cholangiocarcinoma.…”

Section: Discussionmentioning

confidence: 99%

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The Role of Immunoglobulin G4 in Outcomes of Primary Sclerosing Cholangitis

Vujasinovic,

Said,

Villard

et al. 2023

JCM

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Introduction: Primary sclerosing cholangitis (PSC) is a chronic, cholestatic liver disease that is characterized by an inflammatory and fibrotic process affecting bile ducts which eventually develops into liver cirrhosis and liver failure. The aim of this study was to investigate serum IgG subclass distribution in patients with PSC and its possible association with PSC outcomes. Patients and methods: We performed a retrospective analysis of 181 patients who had been diagnosed with PSC between January 1970 and December 2015 and followed at our outpatient clinic. Their demographic, immunological, and clinical characteristics were recorded and analyzed. Results: This study included 181 patients with PSC (120 males, 61 females). There was no association between IgGs and the development of autoimmune hepatitis, cirrhosis, cholangiocarcinoma, liver transplantation, inflammatory bowel disease, and colectomy. Patients with elevated IgG4 had statistically significant higher rates of cholangitis (p = 0.02) and endoscopic retrograde cholangiopancreatography (ERCP) (p = 0.009). High IgG4 values were observed in nine patients who underwent ERCP. In these nine patients, on average, IgG4 was evaluated 5 years after ERCP (min 3 days, max 11 years). Subanalysis considering only IgG4 values evaluated before ERCP showed no significant difference but remains significant if we consider IgG4 values after ERCP. Conclusion: Elevated IgG4 in our study showed a possible association with higher rates of cholangitis and ERCP among patients with primary sclerosing cholangitis. It seems that IgGs may be a useful tool for the prediction of outcomes in patients with PSC. A prospective study is necessary, especially to study the trends of IgGs values during disease as well as the role of possible seroconversion.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

The Role of Immunoglobulin G4 in Outcomes of Primary Sclerosing Cholangitis

Vujasinovic,

Said,

Villard

et al. 2023

JCM

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“…IgG4-SC is also a common complication of multifocal biliary strictures and IBD, and therefore, it may be misdiagnosed in some cases ( 161 ). In contrast to PSC, IgG4-SC is a multisystem fibroinflammatory disease that is defined by substantial lymphoplasmacytic infiltration of IgG4-positive cells and increased serum levels of IgG4 ( 162 ). Both the serum IgG4/IgG1 ratio and IgG4/IgG RNA ratio can be used to differentiate between the two conditions ( 163 ).…”

Section: Inflammasomes and Pyroptosis In Aildsmentioning

confidence: 99%

“…serum levels of IgG4 (162). Both the serum IgG4/IgG1 ratio and IgG4/IgG RNA ratio can be used to differentiate between the two conditions (163).…”

Section: Inflammasomes and Pyroptosis In Igg4-scmentioning

confidence: 99%

Inflammasome and pyroptosis in autoimmune liver diseases

et al. 2023

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Autoimmune hepatitis (AIH), primary biliary cholangitis (PBC), primary sclerosing cholangitis (PSC), and IgG4-related sclerosing cholangitis (IgG4-SC) are the four main forms of autoimmune liver diseases (AILDs), which are all defined by an aberrant immune system attack on the liver. Most previous studies have shown that apoptosis and necrosis are the two major modes of hepatocyte death in AILDs. Recent studies have reported that inflammasome-mediated pyroptosis is critical for the inflammatory response and severity of liver injury in AILDs. This review summarizes our present understanding of inflammasome activation and function, as well as the connections among inflammasomes, pyroptosis, and AILDs, thus highlighting the shared features across the four disease models and gaps in our knowledge. In addition, we summarize the correlation among NLRP3 inflammasome activation in the liver-gut axis, liver injury, and intestinal barrier disruption in PBC and PSC. We summarize the differences in microbial and metabolic characteristics between PSC and IgG4-SC, and highlight the uniqueness of IgG4-SC. We explore the different roles of NLRP3 in acute and chronic cholestatic liver injury, as well as the complex and controversial crosstalk between various types of cell death in AILDs. We also discuss the most up-to-date developments in inflammasome- and pyroptosis-targeted medicines for autoimmune liver disorders.

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