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.

scholarly journals Atypical presentation of autoimmune hepatitis–primary sclerosing cholangitis overlap syndrome associated with hypereosinophilia: a case report and review of the literature

2021 ◽  
Vol 15 (1) ◽  
Author(s):  
Behzad Hatami ◽  
Hamide Rahmani Seraji ◽  
Mohaddese fallahi
Keyword(s):  
Magnetic Resonance ◽  
Autoimmune Hepatitis ◽  
Primary Sclerosing Cholangitis ◽  
Peripheral Blood ◽  
Sclerosing Cholangitis ◽  
Magnetic Resonance Cholangiopancreatography ◽  
Biliary Tree ◽  
Overlap Syndrome ◽  
Elevated Liver Enzymes ◽  
Mitochondrial Antibody

Abstract Background Autoimmune hepatitis–primary sclerosing cholangitis overlap syndrome is a form of autoimmune hepatitis with cholestatic features and is characterized by negative anti-mitochondrial antibody and cholangiographic changes on magnetic resonance cholangiopancreatography or endoscopic retrograde cholangiopancreatography. Peripheral blood hypereosinophilia in conjunction with autoimmune hepatitis–primary sclerosing cholangitis overlap syndrome has not been reported yet. Here we present a case of autoimmune hepatitis–primary sclerosing cholangitis overlap syndrome with hypereosinophilia. Case presentation A 33-year-old Iranian man with the fatigue, jaundice, elevated liver enzymes and alkaline phosphatase, and hypereosinophilia was referred to our hospital. Viral and autoimmune hepatitis were excluded, and secondary workups for hypereosinophilia were all negative. Magnetic resonance cholangiopancreatography showed beaded appearance of intra- and extrahepatic biliary tree, and liver biopsy revealed interface hepatitis. Therefore, the diagnosis of autoimmune hepatitis–primary sclerosing cholangitis overlap syndrome was made, and prednisolone, azathioprine, and ursodeoxycholic acid was initiated. His jaundice and peripheral blood eosinophilia resolved after 2 weeks, and he became completely asymptomatic. Conclusion Eosinophils might contribute to the clinical presentation and disease complications.

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 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.

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

scholarly journals Case Report: Acute presentation of autoimmune hepatitis in a male patient

F1000Research ◽  
2021 ◽  
Vol 10 ◽  
pp. 406
Author(s):  
Aya Hammami ◽  
Khouloud Ben Abdessalem ◽  
Sarra Mestiri ◽  
Nour Elleuch ◽  
Wafa Dahmani ◽  
...  
Keyword(s):  
Liver Disease ◽  
Autoimmune Hepatitis ◽  
Male Patient ◽  
Therapeutic Option ◽  
Rare Condition ◽  
Hepatic Function ◽  
Chronic Liver ◽  
Primary Biliary Cholangitis ◽  
Serologic Marker ◽  
Mitochondrial Antibody

Introduction: Autoimmune hepatitis (AIH) is one of the major immune mediated chronic liver diseases.  It typically affects young and middle-aged females. Acute liver failure (ALF) is an unusual initial form of presentation of AIH and is particularly rare in male patients. Consequently, the clinical characteristics and optimal management of this entity remain poorly defined. Patients with AIH sometimes present features of the spectrum of primary biliary cholangitis (PBC), simultaneously or consecutively, suggesting the diagnosis of overlap syndrome (OS) PBC- AIH. Data concerning PBC-AIH has been scarcely published and mainly comprises small retrospective studies. Case presentation: Herein, we report the case of a 40-year-old man with no history of any chronic liver disease, who presented with ALF. After carrying out extensive etiological screening, we suspected him of having ALF due to auto-immune liver disease namely AIH. The positivity of anti-mitochondrial antibody (AMA) which is a significant serologic marker of PBC, suggested a diagnosis of OS PBC- AIH. Since urgent liver transplantation could not be performed in our country (Tunisia), the only available therapeutic option was the administration of corticosteroids. During the two years of follow up and treatment with ursodeoxycholic acid, azathioprine and a low dose of prednisolone, our patient is still asymptomatic with normal hepatic function tests. Conclusion: ALF due to AIH in a male patient is a very rare condition. The diagnosis should be considered in all patients with acute hepatitis of undetermined etiology. Corticosteroids were an effective and lifesaving therapeutic option. The association of AIH and PBC features could suggest an OS.

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