scholarly journals Dysregulation of Circulating FGF19 and Bile Acids in Primary Biliary Cholangitis-Autoimmune Hepatitis Overlap Syndrome

2020 ◽  
Vol 2020 ◽  
pp. 1-9
Author(s):  
Zhanyi Li ◽  
Yu Liu ◽  
Fangji Yang ◽  
Jiahui Pang ◽  
Yuankai Wu ◽  
...  
Keyword(s):  
Autoimmune Hepatitis ◽  
Bile Acids ◽  
Overlap Syndrome ◽  
Primary Biliary Cholangitis ◽  
Strongly Correlated ◽  
Adequate Treatment ◽  
End Stage ◽  
Fibroblast Growth Factor 19 ◽  
Total Bile Acids

Background. Primary biliary cholangitis-autoimmune hepatitis overlap syndrome (PBC-AIH OS), which exhibits features between autoimmune hepatitis and cholestasis, is a common condition and usually shows a progressive course toward cirrhosis and liver failure without adequate treatment. Synthesis of bile acids (BAs) plays an important role in liver injury in cholestasis, and the process is regulated by fibroblast growth factor 19 (FGF19). The overall role of circulating FGF19 in BA synthesis and PBC-AIH OS requires further investigation. Methods. We analyzed BA synthesis and correlated clinical parameters with serum BAs and FGF19 in 35 patients with PBC-AIH OS. Serum concentrations of 7alpha-hydroxycholest-4-en-3-one (C4) were used to quantify the synthesis of BA directly. Results. Serum FGF19 levels were higher, while C4 levels were substantially lower in PBC-AIH OS patients than those in healthy controls. Circulating FGF19 levels strongly correlated with C4 (r=−0.695, p<0.0001), direct bilirubin (r=0.598, p=0.0001), and total bile acids (r=0.595, p=0.002). Moreover, circulating FGF19 levels strongly correlated with the model for end-stage liver disease score (r=0.574, p=0.0005) and Mayo risk score (r=0.578, p=0.001). Conclusions. Serum FGF19 is significantly increased in patients with PBC-AIH OS, while BA synthesis is suppressed. Circulating FGF19 primarily controls the regulation of BA synthesis in response to cholestasis and under cholestatic conditions. Therefore, modulation of circulating FGF19 could provide a promising targeted therapy for patients with PBC-AIH OS.

scholarly journals Association between Circulating Growth Differentiation Factor 15 and Cirrhotic Primary Biliary Cholangitis

2020 ◽  
Vol 2020 ◽  
pp. 1-11
Author(s):  
Zhanyi Li ◽  
Yu Liu ◽  
Xiangyong Li ◽  
Yuankai Wu ◽  
Fangji Yang ◽  
...  
Keyword(s):  
Serum Levels ◽  
Response To Treatment ◽  
Primary Biliary Cholangitis ◽  
Strongly Correlated ◽  
Weight Changes ◽  
Adequate Treatment ◽  
Growth Differentiation Factor 15 ◽  
Reactive Protein ◽  
Differentiation Factor ◽  
End Stage

Primary biliary cholangitis (PBC) is a common condition that usually shows a progressive course towards cirrhosis without adequate treatment. Growth differentiation factor 15 (GDF15) plays multiple roles in various pathological conditions. The overall role of circulating GDF15 in cirrhotic PBC requires further investigation. Twenty patients with cirrhotic PBC, 26 with non-cirrhotic PBC, and 10 healthy subjects were enrolled between 2014 and 2018, and the serum levels of GDF15 were measured via enzyme immunoassay. The correlations between serum GDF15, weight, biochemical parameters, and the prognosis were analysed. Serum levels of GDF15 were significantly higher in cirrhotic PBC patients than in non-cirrhotic PBC patients or healthy controls ( p = 0.009 and p < 0.001 , respectively). The circulating GDF15 levels strongly correlated with weight changes ( r = − 0.541 , p = 0.0138 ), albumin ( r = − 0.775 , p < 0.0001 ), direct bilirubin ( r = − 0.786 , p < 0.0001 ), total bile acids ( r = 0.585 , p = 0.007 ), and C-reactive protein ( r = 0.718 , p = 0.0005 ). Moreover, circulating GDF15 levels strongly correlated with the Mayo risk score ( r = 0.685 , p = 0.0009 ) and Model for End-stage Liver Disease score ( r = 0.687 , p = 0.0008 ). Determined by the area under the receiver operating characteristic curves, the overall diagnostic accuracies of GDF15 were as follows: cirrhosis = 0.725 (>3646.55 pg/mL, sensitivity: 70.0%, specificity: 69.2%), decompensated   cirrhosis = 0.956 (>4073.30 pg/mL, sensitivity: 84.62%, specificity: 100%), and cirrhotic biochemical non-responders = 0.835 (>3479.20 pg/mL, sensitivity: 71.43%, specificity: 92.31%). GDF15 may be a useful and integrated biochemical marker to evaluate not only the disease severity and prognosis but also the nutrition and response to treatment of cirrhotic PBC patients, and its overall performance is satisfactory. Therapy targeting GDF15 is likely to benefit cirrhotic PBC patients and is worth further research.

scholarly journals Autoimmune Hepatitis and Primary Biliary Cholangitis Overlap in a Patient with Systemic Lupus Erythematosus and Rheumatoid Arthritis Overlap (Rhupus) - An Unusual Association

2022 ◽  
Vol 12 (1) ◽  
pp. 92-98
Author(s):  
Anu Yarky ◽  
Priyesh Sharma ◽  
Vipan Kumar
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Autoimmune Hepatitis ◽  
Lupus Erythematosus ◽  
Treatment Plan ◽  
Liver Function Tests ◽  
Overlap Syndrome ◽  
Primary Biliary Cholangitis ◽  
Systemic Lupus ◽  
Adequate Treatment ◽  
Clinical Presentations

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.

scholarly journals Primary biliary cholangitis‐autoimmune hepatitis overlap syndrome: Case report and literature review

2021 ◽  
Author(s):  
Chantelli Iamblaudiot Razafindrazoto ◽  
Andry Lalaina Rinà Rakotozafindrabe ◽  
Nitah Harivony Randriamifidy ◽  
Anjaramalala Sitraka Rasolonjatovo ◽  
Tovo Harimanana Rabenjanahary ◽  
...  
Keyword(s):  
Case Report ◽  
Literature Review ◽  
Autoimmune Hepatitis ◽  
Overlap Syndrome ◽  
Primary Biliary Cholangitis

scholarly journals Autoimmune Hepatitis–Primary Biliary Cholangitis Overlap Syndrome Triggered by COVID-19

2021 ◽  
Author(s):  
Balraj Singh ◽  
Parminder Kaur ◽  
Michael Maroules
Keyword(s):  
Autoimmune Hepatitis ◽  
Multiple Organ ◽  
Overlap Syndrome ◽  
Beta Thalassemia ◽  
Primary Biliary Cholangitis ◽  
Smooth Muscle Antibody ◽  
Elevated Liver Enzymes ◽  
Thalassemia Minor ◽  
Wide Range ◽  
Mitochondrial Antibody

COVID-19, caused by severe acute respiratory syndrome coronavirus 2 infection, has caused the ongoing global pandemic. Initially considered a respiratory disease, it can manifest with a wide range of complications (gastrointestinal, neurological, thromboembolic and cardiovascular) leading to multiple organ dysfunction. A range of immune complications have also been described. We report the case of a 57-year-old man with a medical history of hypertension, prediabetes and beta thalassemia minor, who was diagnosed with COVID-19 and subsequently developed fatigue and arthralgias, and whose blood work showed hyperferritinemia, elevated liver enzymes (AST/ALT/GGT), hypergammaglobulinemia, anti-smooth muscle antibody, anti-mitochondrial antibody, and anti-double-stranded DNA antibodies. The patient was diagnosed with autoimmune hepatitis–primary biliary cholangitis overlap syndrome triggered by COVID-19. To our knowledge, this is the first such case reported.

Primary Biliary Cholangitis- Autoimmune Hepatitis Overlap Syndrome

2018 ◽  
Vol 85 (9) ◽  
pp. 803-805
Author(s):  
Pratin Bhatt ◽  
Deepak Kumar Gupta ◽  
Nitin Ramani ◽  
Pratik Tibdewal ◽  
Abhishek Sadalage ◽  
...  
Keyword(s):  
Autoimmune Hepatitis ◽  
Overlap Syndrome ◽  
Primary Biliary Cholangitis

443 Association of GPBAR1 Gene Variant With Satiation and Circulating Fibroblast Growth Factor-19 Supports a Role of Bile Acids in Obesity

2015 ◽  
Vol 81 (5) ◽  
pp. AB146-AB147
Author(s):  
Andres Acosta ◽  
Nelson Valentin Feliciano ◽  
Paula Carlson ◽  
Deborah J. Eckert ◽  
Jessica O'Neill ◽  
...  
Keyword(s):  
Growth Factor ◽  
Bile Acids ◽  
Fibroblast Growth Factor ◽  
Fibroblast Growth ◽  
Gene Variant ◽  
Fibroblast Growth Factor 19
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