Autoimmune Hepatitis Overlap Syndrome Research Articles

Liver Failure in Autoimmune Hepatitis Overlap Syndrome With Primary Biliary Cholangitis: A Case Report

Liver Failure in Autoimmune Hepatitis Overlap Syndrome With Primary Biliary Cholangitis: A Case Report

Clinical complexity unveiled: A case of primary biliary cholangitis autoimmune hepatitis overlap syndrome

Overlap syndrome with primary biliary cholangitis and autoimmune hepatitis (PBC-AIH) remain uncommon liver autoimmune-related disorders. Diagnosis is based on clinical, biochemical, serological, and histological findings. Early diagnosis of the disease will give the best outcome even though evidence-proven treatment is still unavailable to tackle this disease entity. Here, we present a case of a Sri Lankan woman diagnosed with PBC-AIH overlap syndrome. She presented with symptoms of jaundice and pruritus. Extensive investigations, including liver biopsy were instrumental in confirming the diagnosis.

Thirteen-Year Follow-Up of a Patient with Liver Cirrhosis Resulting from the Overlap Syndrome of Autoimmune Hepatitis and Primary Biliary Cholangitis: Severe COVID-19 and Liver Transplantation

Aim: to present the difficulties of long-term management of a patient with liver cirrhosis in the outcome of overlap syndrome (autoimmune hepatitis and primary biliary cholangitis) who suffered from severe COVID-19 infection.Key points. The diagnosis of liver cirrhosis as an outcome of overlap syndrome (autoimmune hepatitis and primary biliary cholangitis) was established at the patient’s age of 33 years. At the age of 40, the patient became pregnant for the first time, the pregnancy proceeded well, and a cesarean section was performed at 36 weeks. At the age of 45, the patient suffered a severe new coronavirus infection, followed by decompensation of liver cirrhosis, which required liver transplantation 4 months after COVID-19, followed by a favorable postoperative course.Conclusion. This clinical case demonstrates the successful onset and outcome of pregnancy in a patient with liver cirrhosis in the outcome of overlap syndrome (autoimmune hepatitis and primary biliary cholangitis). The pronounced activity of the disease after severe new coronavirus infection required liver transplantation with successful outcome.

S1497 Clinical Characteristics and Outcomes of Patients with Primary Biliary Cholangitis–Autoimmune Hepatitis Overlap Syndrome – A Multicentric Cohort Study

S1497 Clinical Characteristics and Outcomes of Patients with Primary Biliary Cholangitis–Autoimmune Hepatitis Overlap Syndrome – A Multicentric Cohort Study

Overlap syndrome of autoimmune hepatitis, Primary biliary cholangitis leading to decompensated cirrhosis in a patient of systemic lupus erythematosus: a case report of complex concomitant autoimmune disease.

Background and Aim: Overlap syndrome is a spectrum of clinical manifestations of autoimmune hepatitis (AIH) and Primary Biliary Cholangitis (PBC).it’s unusual to occur overlap syndrome ( AIH &PBC) in a patient with Systemic lupus erythematosus.(SLE)

A Case of Autoimmune Hepatitis/Primary Biliary Cholangitis Overlap Syndrome during Treatment with Brodalumab for Generalized Pustular Psoriasis

Psoriasis is a chronic inflammatory skin disease characterized by accelerated tumor necrosis factor-α (TNF-α) /interleukin (IL) -23/IL-17 axis, epidermal hyperproliferation, and dysregulated differentiation. Psoriasis is occasionally associated with autoimmune liver diseases such as autoimmune hepatitis (AIH) or primary biliary cholangitis (PBC), caused by autoimmunity against hepatocyte- or cholangiocyte-specific autoantigens, respectively. Overlap syndrome is a condition in which patients have features of both AIH and PBC. It has been reported that AIH, PBC, or the overlap syndrome can be triggered by certain drug therapies. A 65-year-old Japanese man developed increased serum levels of aspartate and alanine aminotransferases, and positive anti-nuclear and anti-mitochondrial M2 antibodies, along with neutropenia, at 4 weeks of treatment with an anti-IL-17 receptor A antibody brodalumab for generalized pustular psoriasis. Histological evaluation of the liver revealed interface hepatitis and non-suppurative destructive cholangitis, which is compatible with the overlap syndrome of AIH and PBC. This is the first case of AIH/PBC overlap syndrome during treatment with brodalumab for generalized pustular psoriasis. The relationship between brodalumab and AIH/PBC overlap syndrome should be further elucidated. The risk of autoimmune liver diseases in patients with psoriasis treated with brodalumab should be carefully considered.

Clinical Features and Outcomes of Primary Sclerosing Cholangitis in the Highly Admixed Brazilian Population.

Background Primary sclerosing cholangitis (PSC) is associated with a broad phenotypic spectrum in different populations from diverse ethnic and racial backgrounds. This study aimed to describe the clinical characteristics and outcomes of PSC in a multicenter cohort of patients from Brazil. Methods Data from the Brazilian Cholestasis Study Group were retrospectively reviewed to assess demographic information and clinical characteristics of PSC, as well as the outcomes, such as transplantation-free survival. Results This cohort included 210 patients. After excluding 33 (15.7%) patients with PSC and overlap syndrome of autoimmune hepatitis, 177 (97 males, median age 33 (21–42) years) with clear-cut PSC were eligible for this study. Most of the patients (n = 139, 78.5%) were symptomatic, and 104 (58.7%) had advanced PSC at the time of diagnosis. Concurrent inflammatory bowel disease was observed in 78 (58.6%) of the investigated patients (n = 133), and most of them had ulcerative colitis (n = 61, 78.2%). The 1- and 5-year survival free of liver transplantation or death were 92.3 ± 2.1% and 66.9 ± 4.2%, respectively, and baseline advanced PSC, pruritus, and elevated bilirubin levels were independent risk factors for the composite adverse outcome. Females were significantly older and had lower bilirubin levels than males at baseline, but survival was not associated with sex. Approximately 12.4% (n = 22) of patients with PSC died, and 32.8% (n = 58) underwent liver transplantation at a median follow-up time of 5.3 and 3.2 years. Conclusion Multiethnic Brazilian PSC patients exhibited a less pronounced male predominance and a lower frequency of inflammatory bowel disease than Caucasians. Adverse outcomes were more frequent, probably due to advanced disease at baseline.

Value and risk of percutaneous liver biopsy in patients with cirrhosis and clinical suspicion of autoimmune hepatitis

ObjectiveThe decision regarding whether to perform a liver biopsy in patients with cirrhosis and clinically suspected autoimmune hepatitis (AIH) remains a challenge. This study aimed to assess the utility and...

Characteristics and Inpatient Outcomes of Primary Biliary Cholangitis and Autoimmune Hepatitis Overlap Syndrome.

Background and AimsPrimary biliary cholangitis (PBC) and autoimmune hepatitis (AIH) are hepatobiliary diseases of presumed immune-mediated origin that have been shown to overlap. The aim of this retrospective trial was to use national data to examine the characteristics and outcomes of patients hospitalized with overlapping PBC and AIH (PBC/AIH).MethodsThe National Inpatient Sample was used to identify hospitalized adult patients with PBC, AIH, and PBC/AIH from 2010 to 2014 by International Classification of Diseases-Ninth Edition Revision codes; patients with hepatitis B virus and hepatitis C virus infection were excluded. Primary outcomes measures were in-hospital outcomes that included mortality, respiratory failure, septic shock, length of stay, and total hospital charges. Secondary outcomes were the clinical characteristics of PBC/AIH, including the comorbid extrahepatic autoimmune disease pattern and complications of cirrhosis.ResultsA total of 3,478 patients with PBC/AIH were included in the study. PBC/AIH was associated with higher rates of Sjögren’s syndrome (p<0.001; p<0.001), lower rates of Crohn’s disease (p<0.05; p<0.05), and higher rates of cirrhosis-related complications when compared to PBC or AIH alone. There were similar rates of mortality between the PBC/AIH, PBC, and AIH groups. The PBC/AIH group had higher rates of septic shock when compared to the PBC group (p<0.05) and AIH group (p<0.05) after adjusting for possible confounders.ConclusionsPBC/AIH is associated with a lower rate of Crohn’s disease, a higher rate of Sjögren’s syndrome, higher rates of cirrhosis-related complications, and significantly increased risk of septic shock compared to PBC and AIH individually.

FibroTouch diagnostic efficiency and influencing factors for liver fibrosis in autoimmune liver diseases

FibroTouch (FT) has been used widely in clinic. Studies of the FT diagnostic efficiency and influencing factors of liver stiffness measurement (LSM) of liver fibrosis in autoimmune liver diseases (AILD) have shown that FT has a good diagnostic efficiency and accuracy, especially in AIH. However, for patients with primary biliary cholangitis and overlap syndrome of autoimmune hepatitis, FT results should be vigilant. In addition, the diagnostic efficiency of FT. Notably, when using FT to diagnose AILD, the value of elastic stiffness depends on five influencing factors, such as, age, total bile acid, international standardized ratio, FIB-4 index, and prothrombin time.

S3537 Overlap Syndrome of Autoimmune Hepatitis and Primary Biliary Cirrhosis Diagnosed in a Man

S3537 Overlap Syndrome of Autoimmune Hepatitis and Primary Biliary Cirrhosis Diagnosed in a Man

Primary sclerosing cholangitis and autoimmune hepatitis overlap syndrome associated with inflammatory bowel disease: A case report and systematic review.

BACKGROUNDA previously healthy 22-year-old woman presented with abdominal pain and jaundice. She had a reagent antinuclear factor (1:640, with a homogeneous nuclear pattern) and hypergammaglobulinemia (2.16 g/dL). Anti-smooth muscle, anti-mitochondrial and anti-liver-kidney microsomal antibody type 1 antibodies were negative. Magnetic resonance cholangiography showed a cirrhotic liver with multiple focal areas of strictures of the intrahepatic bile ducts, with associated dilations. Liver biopsy demonstrated periportal necroinflammatory activity, plasmocyte infiltration and advanced fibrosis. Colonoscopy showed ulcerative pancolitis and mild activity (Mayo score 1), with a spared rectum. Treatment with corticosteroids, azathioprine, ursodeoxycholic acid and mesalamine was initiated, with improvement in laboratory tests. The patient was referred for a liver transplantation evaluation.AIMTo report the case of a female patient with autoimmune hepatitis and primary sclerosing cholangitis (PSC) overlap syndrome associated with ulcerative colitis and to systematically review the available cases of autoimmune hepatitis and PSC overlap syndrome.METHODSIn accordance with preferred reporting items for systematic reviews and meta-analysis protocols guidelines, retrieval of studies was based on medical subject headings and health sciences descriptors, which were combined using Boolean operators. Searches were run on the electronic databases Scopus, Web of Science, MEDLINE (PubMed), Biblioteca Regional de Medicina, Latin American and Caribbean Health Sciences Literature, Cochrane Library for Systematic Reviews and Opengray.eu. Languages were restricted to English, Spanish and Portuguese. There was no date of publication restrictions. The reference lists of the studies retrieved were searched manually.RESULTSThe search strategy retrieved 3349 references. In the final analysis, 44 references were included, with a total of 109 cases reported. The most common clinical finding was jaundice and 43.5% of cases were associated with inflammatory bowel disease. Of these, 27.6% were cases of Crohn’s disease, 68% of ulcerative colitis, and 6.4% of indeterminate colitis. Most patients were treated with steroids. All-cause mortality was 3.7%.CONCLUSIONPSC and autoimmune hepatitis overlap syndrome is generally associated with inflammatory bowel disease and has low mortality and good response to treatment.

Serum Matrix Metalloproteinase 7 Is a Diagnostic Biomarker of Biliary Injury and Fibrosis in Pediatric Autoimmune Liver Disease.

In autoimmune liver disease (AILD), including autoimmune hepatitis (AIH), primary sclerosing cholangitis (PSC), and overlap syndrome of AIH and PSC (ASC), the presence of biliary injury portends a worse prognosis. We studied serum matrix metalloproteinase 7 (sMMP7) as a biomarker for pediatric sclerosing cholangitis (SC). We prospectively enrolled 54 children (median age, 16 years) with AILD (AIH, n = 26; ASC, n = 16; and PSC, n = 12) at our center. The sMMP7 concentrations were higher in patients with SC compared to those without cholangiopathy (P < 0.001). An sMMP7 concentration >23.7 ng/mL had a sensitivity and specificity of 79% and 96%, respectively, and outperformed alkaline phosphatase (ALP) and gamma‐glutamyltransferase (GGT) in segregating patients with SC. Serum concentrations correlated with liver gene expression levels for MMP7 (r = 0.70; P < 0.001). Using immunofluorescence, MMP7 was localized primarily to the cholangiocytes of patients with SC. In 46 subjects with liver biopsy available for blinded review, elevation in sMMP7 concentrations segregated with the presence of lymphocytic and neutrophilic cholangitis and periductal fibrosis and correlated with Ishak, Ludwig, and Nakanuma scoring systems. Liver stiffness measured by magnetic resonance elastography also correlated with sMMP7 concentrations (r = 0.56; P < 0.01). Using magnetic resonance cholangiopancreatography plus (MRCP+), sMMP7 in 34 patients correlated with the number of biliary dilatations (r = 0.54; P < 0.01) and strictures (r = 0.56; P < 0.01). MMP7 as a marker of biliary injury was validated in an independent cohort of children with ulcerative colitis. Higher sMMP7 concentrations also correlated with a history of SC‐related complication. Conclusion: MMP7 is a promising biomarker for pediatric SC that diagnostically outperforms ALP and GGT. sMMP7 may directly reflect biliary injury and fibrosis, the main drivers of disease progression in SC.

English

English

HLA-DRB1 GENE POLYMORPHISMS IN PEDIATRIC PATIENTS WITH TYPE 1 AUTOIMMUNE HEPATITIS AND TYPE 1 AUTOIMMUNE HEPATITIS OVERLAP SYNDROME WITH AUTOIMMUNE CHOLANGITIS.

Autoimmune hepatitis (AIH) is a rare chronic inflammatory liver disease associated with a loss of immunological tolerance to self-antigens. Susceptibility to AIH is partially determined by the presence of genes related to human leukocyte antigen (HLA), mainly allelic variants of DRB1. The purpose of this study was to investigate the frequencies of the polymorphisms in HLA-DRB1 gene in children and adolescents with type 1 AIH and type 1 AIH overlap syndrome with autoimmune cholangitis (overlap syndrome, OS) in comparison to healthy sex and age-matched individuals (control group). This is a cross-sectional study of 25 pediatric patients diagnosed with type 1 AIH and 18 with OS. Fifty-seven healthy individuals were included as controls. The polymorphisms of the HLA-DRB1 gene were evaluated by PCR and included HLA-DRB1*03, HLA-DRB1*04, HLA-DRB1*07, and HLA-DRB1*13. Our results showed that the presence of the allele HLA-DRB1*13 increased the chance of autoimmune cholangitis (OR=3.96, CI 1.07 to 14.61, P=0.04). The HLA-DRB1*04 and HLA- DRB1*07 have no association with the AIH and autoimmune cholangitis in a young sample. This work demonstrates an association of the main polymorphisms in the HLA-DRB1 gene to AIH with or without cholangitis in a Brazilian sample.

Tu1531 – Primary Biliary Cholangitis (PBC)-Autoimmune Hepatitis (AIH) Overlap Syndrome: Characteristics and Response to Obeticholic Acid (OCA) in Target-PBC, a Diverse, Large United States (US) Real-World Cohort

Tu1531 – Primary Biliary Cholangitis (PBC)-Autoimmune Hepatitis (AIH) Overlap Syndrome: Characteristics and Response to Obeticholic Acid (OCA) in Target-PBC, a Diverse, Large United States (US) Real-World Cohort

Case Report and Review of Literature: An Overlap Syndrome of Autoimmune Hepatitis and Systemic Lupus Erythematous

Case Report and Review of Literature: An Overlap Syndrome of Autoimmune Hepatitis and Systemic Lupus Erythematous

New scoring classification for primary biliary cholangitis-autoimmune hepatitis overlap syndrome.

Autoimmune hepatitis (AIH) and primary biliary cholangitis (PBC) are two major immune‐mediated chronic liver diseases. Overlap syndrome (OS) is diagnosed if patients have features of both AIH and PBC; however, there is no consensus on the definition or diagnostic criteria for OS. Here, we report a new scoring classification for OS and evaluate its usefulness. This new scoring classification was developed by modifying the International Autoimmune Hepatitis Group classification by selecting histologic features of AIH and PBC along with modifications of biochemical and immunologic characteristics. We evaluated 272 patients with chronic liver disease, including 105 with AIH, 102 with PBC, and 65 with OS. The best performance for the diagnosis of OS was noted among patients with an overlap score of ≥21 who had a sensitivity of 98.5%, a specificity of 92.8%, a positive predictive value of 81.0%, and a negative predictive value of 99.5%. By using a cut‐off score of 21, 64 (98.5%) patients were diagnosed with OS as opposed to 9 (8.8%) and 6 (5.7%) with PBC and AIH, respectively. All patients with OS had an aggregate score of >19, whereas most patients with PBC or AIH scored <19, making this a safe discriminatory cut‐off point against OS. Conclusion: The new scoring system for the diagnosis of OS has a high sensitivity and specificity for scores ≥21, while a score <19 suggests a diagnosis other than OS. This classification can identify patients and diagnose OS with a reasonable amount of accuracy and may be superior to current OS scoring systems in detecting mild forms of OS. (Hepatology Communications 2018;2:245‐253)

Primary Biliary Cholangitis- Autoimmune Hepatitis Overlap Syndrome

Primary Biliary Cholangitis- Autoimmune Hepatitis Overlap Syndrome

Overlap Syndrome of Autoimmune Hepatitis and Primary Biliary Cirrhosis: A Clinical Enigma

“Overlap syndrome” is a term used to describe variant forms of autoimmune hepatitis (AIH) which present with characteristics of AIH and primary biliary cirrhosis (PBC) or primary sclerosing cholangitis (PSC). Patients with overlap syndrome present with both hepatitic and cholestatic serum liver test and the clinical, biochemical, and histological features of these autoimmune diseases are overlapped. Thus, it is difficult to appreciate overlap syndrome as an actual diagnostic entity. AIH-PBC is the most common form of overlap syndrome, affecting almost 10% of adults with AIH or PBC. Transitions from PBC to AIH-PBC overlap syndrome have also been reported. Ursodeoxycholic acid is usually combined with immunosuppressive therapy but end-stage disease requires liver transplantation. We present a case of Overlap Syndrome (AIHPBC) in a 16 year old girl who presented to us with features of obstructive jaundice. She had six months history of generalised itching, yellow discoloration of skin and urine, decreased appetite and intermittent abdominal pain. Liver function test revealed conjugated hyperbilirubinemia, with moderate elevation of liver enzymes. Antinuclear, anti-muscle M2 and anti-glycoprotein 210 antibodies were positive. Liver biopsy showed features of primary biliary cirrhosis. The patient responded to ursodeoxycholic acid and immunosuppression.