primary biliary cholangitis
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Life ◽  
2022 ◽  
Vol 12 (1) ◽  
pp. 124
Noriko Ishii-Kitano ◽  
Hirayuki Enomoto ◽  
Takashi Nishimura ◽  
Nobuhiro Aizawa ◽  
Yoko Shibata ◽  

Inflammatory pseudotumor (IPT) of the liver is a rare benign disease. IPTs generally develop as solitary nodules, and cases with multiple lesions are uncommon. We herein report a case of multiple IPTs of the liver that spontaneously regressed. A 70-year-old woman with a 10-year history of primary biliary cholangitis and rheumatoid arthritis visited our hospital to receive a periodic medical examination. Abdominal ultrasonography revealed multiple hypoechoic lesions, with a maximum size of 33 mm, in the liver. Contrast-enhanced computed tomography revealed low-attenuation areas in the liver with mild peripheral enhancement at the arterial and portal phases. We first suspected metastatic liver tumors, but fluorodeoxyglucose positron emission tomography, magnetic resonance imaging and contrast-enhanced ultrasonography suggested the tumors to be inconsistent with malignant nodules. A percutaneous biopsy showed shedding of liver cells and abundant fibrosis with infiltration of inflammatory cells. Given these findings, we diagnosed the multiple tumors as IPTs. After careful observation for two months, the tumors almost vanished spontaneously. Physicians should avoid a hasty diagnosis of multiple tumors based solely on a few clinical findings, and a careful assessment with various imaging modalities should be conducted.

Katarzyna Trzos ◽  
Natalia Pydyn ◽  
Jolanta Jura ◽  
Jerzy Kotlinowski

AbstractMurine models of human diseases are of outmost importance for both studying molecular mechanisms driving their development and testing new treatment strategies. In this review, we first discuss the etiology and risk factors for autoimmune liver disease, including primary biliary cholangitis, autoimmune hepatitis and primary sclerosing cholangitis. Second, we highlight important features of murine transgenic models that make them useful for basic scientists, drug developers and clinical researchers. Next, a brief description of each disease is followed by the characterization of selected animal models.

2022 ◽  
Vol 2022 ◽  
pp. 1-7
Mingjie Yao ◽  
Leijie Wang ◽  
Jianwen Wang ◽  
Yanna Liu ◽  
Shuhong Liu ◽  

Background. There is lack of reliable serum biomarkers to reflect the severity of liver necroinflammation for those who suffer autoimmune liver diseases (AILDs). In this study, a previously established patient cohort was used to explore the potential of serum Golgi protein 73 (GP73) as a noninvasive marker of AILD-related liver necroinflammation. Methods. Serum GP73 concentration was measured in a retrospective cohort of 168 AILD patients, which included 74 patients with autoimmune hepatitis (AIH) and 94 with primary biliary cholangitis (PBC) who had undergone liver biopsy. Spearman’s correlation and multivariate analysis were used to evaluate the relationship between serum GP73 and liver necroinflammation. A receiver operating characteristic curve was constructed to evaluate the value of GP73 for the prediction of moderate or severe liver necroinflammation. The diagnostic value of serum GP73 was also compared with that of alkaline phosphatase (ALP) in patients with PBC. Histologically, immunohistochemical analysis was performed to assess hepatic GP73 expression. Results. Both the serum level and hepatic tissue expression of GP73 protein were aberrantly elevated and correlated well with the severity of necroinflammation in both AIH ( rho = 0.655 , P < 0.001 ) and PBC ( rho = 0.547 , P < 0.001 ) patients. The results here suggested that serum GP73 could be an independent biomarker to reflect the severity of liver necroinflammation. The AUROCs for GP73 to predict moderate necroinflammation (≥G2) and severe necroinflammation (≥G3) in patients with AIH were 0.828 and 0.832, respectively. Moreover, the AUROCs of serum GP73 for the identification of moderate necroinflammation (≥G2) ( AUROC = 0.820 , P < 0.001 ) and severe necroinflammation (≥G3) ( AUROC = 0.803 , P < 0.001 ) were superior to those of ALP (≥G2: AUROC = 0.607 , P = 0.028 and ≥G3: AUROC = 0.559 , P = 0.357 ) in patients with PBC. Mechanically, interlukin-6 (IL-6), the proinflammatory and prohepatic regenerating cytokine, could transcriptionally upregulate GP73 gene expression. Conclusion. Serum GP73 is a potential noninvasive biomarker to evaluate the severity of liver necroinflammation in patients with AILDs.

2022 ◽  
Vol 12 (1) ◽  
pp. 92-98
Anu Yarky ◽  
Priyesh Sharma ◽  
Vipan Kumar

The diagnosis of overlap syndrome involving systemic lupus erythematosus (SLE) and autoimmune hepatitis (AIH) isn’t easily established due to its similar clinical presentations and biochemical features to those of lupus hepatitis. The term overlap syndrome is typically utilized in the context of overlap of autoimmune hepatitis with PSC (primary sclerosing cholangitis) or PBC (primary biliary cholangitis). Few rare cases of AIH complicated by SLE are reported within the literature. Overlapping of SLE and AIH should be suspected when patients with SLE have abnormal liver function tests or AIH patients present with a rash. Liver biopsy plays a really important role in establishing the medical diagnosis of SLE with liver impairment or overlap with AIH. The prompt diagnosis and adequate treatment plan can improve the disease outcome. Key words: autoimmune hepatitis, primary biliary cholangitis, systemic lupus erythematosus, overlap syndrome.

2022 ◽  
Vol 126 ◽  
pp. 102775
Yuki Hitomi ◽  
Yoshihiro Aiba ◽  
Kazuko Ueno ◽  
Nao Nishida ◽  
Yosuke Kawai ◽  

2021 ◽  
Vol 2021 ◽  
pp. 1-16
Yuxin Luo ◽  
Jinbo Guo ◽  
Wenxiu Jia ◽  
Mengyao Wu ◽  
Fengrong Yin ◽  

Nonalcoholic steatohepatitis (NASH) is a progressive, chronic liver disease worldwide which imposes a large economic burden on society. M1/M2 macrophage balance destruction and recruitment of mononuclear immune cells to the liver play critical roles in NASH. Several studies have shown that the expression of TNF-like ligand 1 aberrance (TL1A) increased in macrophages associated with many inflammatory diseases, for example, inflammatory bowel disease, primary biliary cholangitis, and liver fibrosis. One recent research showed that weight, abdominal adipose, and liver leptin, one of the critical fat cytokines, were reduced in TL1A knockout mice. However, the functional and molecular regulatory mechanisms of TL1A on macrophage polarization and recruitment in NASH have yet to be clarified. The authors found that high fructose high fat diet and methionine-choline deficiency diet induced the expression of TL1A in macrophages of liver tissue from murine NASH models. Myeloid-specific TL1A overexpressed mice showed exacerbated steatohepatitis with increased hepatic lipid accumulation, inflammation, liver injury, and apoptosis. M1 macrophages’ infiltration and the production of proinflammatory and chemotactic cytokines increased in liver of NASH mouse models with myeloid-specific TL1A overexpressed. Furthermore, this paper revealed that bone marrow-derived macrophages and Kupffer cells with overexpression of TL1A exacerbated the lipid accumulation and expression of proinflammatory factors in the murine primary hepatocytes after free fatty acid treatment in vitro. In conclusion, TL1A-mediated M1-type macrophage polarization and recruitment into the liver promoted steatohepatitis in murine NASH.

10.52586/5042 ◽  
2021 ◽  
Vol 26 (12) ◽  
pp. 1480-1492
Naohiro Nakamura ◽  
Katsunori Yoshida ◽  
Rinako Tsuda ◽  
Miki Murata ◽  
Takashi Yamaguchi ◽  

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