scholarly journals The risk of liver cancer in autoimmune liver diseases

2019 ◽  
Vol 11 ◽  
pp. 175883591986191 ◽  
Author(s):  
Ana Lleo ◽  
Ynto S. de Boer ◽  
Rodrigo Liberal ◽  
Massimo Colombo
Keyword(s):  
Early Diagnosis ◽  
Liver Diseases ◽  
Close Association ◽  
Fatal Outcome ◽  
Primary Biliary Cholangitis ◽  
Primary Malignancy ◽  
Abdominal Ultrasonography ◽  
Systematic Analysis ◽  
Acid Therapy ◽  
Autoimmune Liver Diseases

Hepatocellular carcinoma (HCC), the dominant primary malignancy of the liver, has almost invariably a fatal outcome that can be averted only by early diagnosis and treatment. While the close association of HCC with chronic viral hepatitis and alcohol abuse has impacted favourably on screening and treatment of this deadly tumour, at the same time it has long obscured the etiologic role of autoimmune liver diseases. Recently, a systematic analysis of 25 published cohorts disclosed a 3.1 × 1000 patients/year incidence of HCC in autoimmune hepatitis patients that tripled in those with cirrhosis. HCC is also a sequela of primary biliary cholangitis, where the incidence is more relevant in males, those with advanced liver disease and nonresponders to ursodeoxycholic acid therapy. Cholangiocarcinoma (CCA), the second ranking primary cancer of the liver, is also on the rise with its intrahepatic pattern, in part reflecting an association with chronic liver diseases of diverse aetiology. In the USA and northern Europe, perihilar CCA is a frequent complication of primary sclerosing cholangitis, a cholestatic disorder thought to be immune mediated. International Guidelines clearly recommend HCC screening with abdominal ultrasonography every 6 months in autoimmune cirrhotic patients. While surveillance of patients with autoimmune liver disorders who are at risk of HCC affects both early diagnosis and radical therapy of this tumour, this is not the case for CCA, where early diagnosis is challenged by the lack of sensitive and accurate tests for screening.

scholarly journals Current Trends and Characteristics of Hepatocellular Carcinoma in Patients with Autoimmune Liver Diseases

Cancers ◽  
2021 ◽  
Vol 13 (5) ◽  
pp. 1023
Author(s):  
Eirini I. Rigopoulou ◽  
George N. Dalekos
Keyword(s):  
Risk Factors ◽  
Hepatocellular Carcinoma ◽  
Liver Diseases ◽  
Treatment Modalities ◽  
Primary Biliary Cholangitis ◽  
Autoimmune Liver Diseases ◽  
Increased Risk ◽  
Liver Cancers ◽  
Medium Risk ◽  
Causes Of Mortality

Hepatocellular carcinoma (HCC), the commonest among liver cancers, is one of the leading causes of mortality among malignancies worldwide. Several reports demonstrate autoimmune liver diseases (AILDs), including autoimmune hepatitis (AIH), primary biliary cholangitis (PBC), and primary sclerosing cholangitis (PSC) to confer increased risk of hepatobiliary malignancies, albeit at lower frequencies compared to other liver diseases. Several parameters have been recognized as risk factors for HCC development in AIH and PBC, including demographics such as older age and male sex, clinical features, the most decisive being cirrhosis and other co-existing factors, such as alcohol consumption. Moreover, biochemical activity and treatment response have been increasingly recognized as prognostic factors for HCC development in AIH and PBC. As available treatment modalities are effective only when HCC diagnosis is established early, surveillance has been proven essential for HCC prognosis. Considering that the risk for HCC is not uniform between and within disease groups, refinement of screening strategies according to prevailing demographic, clinical, and molecular risk factors is mandated in AILDs patients, as personalized HCC risk prediction will offer significant advantage in patients at high and/or medium risk. Furthermore, future investigations should draw attention to whether modification of immunosuppression could benefit AIH patients after HCC diagnosis.

The Association of Two Rare Diseases is Not Rare: Primary Immunodeficiencies and Autoimmune Liver Diseases

2021 ◽  
Author(s):  
Şefika Nur Ayar ◽  
Elif Soyak ◽  
Cem Şimşek ◽  
Deniz Çağdaş ◽  
Yasemin Balaban
Keyword(s):  
Rare Diseases ◽  
Liver Diseases ◽  
Strong Association ◽  
Laboratory Data ◽  
Tertiary Hospital ◽  
Iga Deficiency ◽  
Primary Biliary Cholangitis ◽  
Cross Sectional ◽  
Autoimmune Liver Diseases ◽  
Igm Deficiency

Abstract Purpose: PIDs associates with autoimmune diseases include autoimmune liver diseases (AILD); however, the frequency of PIDs among patients with AILD is unknown. This study aimed to evaluate the strength of the association between AILD and PIDs. Methods: We conducted this single-center, cross-sectional, and descriptive study in a tertiary hospital. We evaluated eighty-two patients with AILD (39 autoimmune hepatitis (AIH), 32 with primary biliary cholangitis (PBC), seven with variant syndromes (VS), and four with primary sclerosing cholangitis (PSC) for the presence of PIDs. We obtained a detailed history of infections, comorbidities, family history, and laboratory data from the files. All patients were evaluated in the immunology department for further examination, and PID diagnoses were made according to ESID (The European Society for Immunodeficiencies) criteria. Results: Out of 82 patients with AILD, PIDs were diagnosed in 18% (15 patients); there were four patients with common variable immunodeficiency (CVID), four with partial IgA deficiency (PIgAD), four with selective IgM deficiency (SIgMD), and three with combined immunodeficiency (CID). PIDs were present in 29% of patients with VS, 25 % of patients with PSC, 23% of patients with AIH, and 9% of patients with PBC. Conclusion: Although PIDs are rare diseases in the general population, they have a strong association with AILD and were detected in one-fifth of the patients. Further research with larger patient groups is needed to evaluate the diagnostic and prognostic impacts of PIDs on AILD.

scholarly journals Variety of lung involvement in autoimmune liver diseases

2018 ◽  
Vol 90 (8) ◽  
pp. 107-112
Author(s):  
L A Akulkina ◽  
M Yu Brovko ◽  
V I Sholomova ◽  
T P Rozina ◽  
A S Yanakayeva ◽  
...  
Keyword(s):  
Liver Diseases ◽  
Primary Biliary Cholangitis ◽  
Extrahepatic Manifestations ◽  
Specific Procedure ◽  
High Resolution Computed Tomography ◽  
Lung Lesions ◽  
Autoimmune Liver Diseases ◽  
Pulmonary Parenchyma ◽  
Lung Vessels

The primary autoimmune liver diseases conventionally include primary biliary cholangitis, primary sclerosing cholangitis and autoimmune hepatitis. Despite of primary autoimmune affection of different parts of the hepatobiliary system, in the recent decades, a lot of data has emerged indicating the presence of extrahepatic manifestations of these diseases, in particular, lung lesions, such as nodular and interstitial changes with possible progression and development of fibrosis and respiratory failure. In case of lungs disease, both pulmonary parenchyma and lung vessels, pleura, and intrathoracic lymph nodes can be involved. The most sensitive and specific procedure to assess the extent of the lung lesions and their evolution is high-resolution computed tomography. Due to the possibility of long-term asymptomatic course of the pulmonary disease with development of irreversible changes in patients with autoimmune liver diseases, it seems reasonable to conduct screening studies aimed at early detection and treatment of lung lesions in this population.

scholarly journals The Microbiome in Autoimmune Liver Diseases: Metagenomic and Metabolomic Changes

2021 ◽  
Vol 12 ◽  
Author(s):  
Yanping Zheng ◽  
Ying Ran ◽  
Hongxia Zhang ◽  
Bangmao Wang ◽  
Lu Zhou
Keyword(s):  
Gut Microbiome ◽  
Metabolic Pathways ◽  
Liver Diseases ◽  
Critical Role ◽  
Short Chain Fatty Acids ◽  
Primary Biliary Cholangitis ◽  
Mechanistic Studies ◽  
Autoimmune Liver Diseases ◽  
Acid Metabolites

Recent studies have identified the critical role of microbiota in the pathophysiology of autoimmune liver diseases (AILDs), including autoimmune hepatitis (AIH), primary biliary cholangitis (PBC), and primary sclerosing cholangitis (PSC). Metagenomic studies reveal significant decrease of gut bacterial diversity in AILDs. Although profiles of metagenomic vary widely, Veillonella is commonly enriched in AIH, PBC, and PSC. Apart from gut microbiome, the oral and bile microbiome seem to be associated with these diseases as well. The functional analysis of metagenomics suggests that metabolic pathways changed in the gut microbiome of the patients. Microbial metabolites, including short-chain fatty acids (SCFAs) and microbial bile acid metabolites, have been shown to modulate innate immunity, adaptive immunity, and inflammation. Taken together, the evidence of host–microbiome interactions and in-depth mechanistic studies needs further accumulation, which will offer more possibilities to clarify the mechanisms of AILDs and provide potential molecular targets for the prevention and treatment in the future.

Recent advances in clinical practice: epidemiology of autoimmune liver diseases

Gut ◽  
2021 ◽  
pp. gutjnl-2020-322362
Author(s):  
Palak J Trivedi ◽  
Gideon M Hirschfield
Keyword(s):  
Liver Diseases ◽  
Organ Transplant ◽  
Population Based ◽  
Incidence Rates ◽  
Asia Pacific ◽  
Primary Biliary Cholangitis ◽  
Disease Epidemiology ◽  
Autoimmune Liver Diseases ◽  
Patient Morbidity ◽  
Patients Experience

Autoimmune liver diseases are chronic inflammatory hepatobiliary disorders that when classically defined encompass three distinctive clinical presentations; primary biliary cholangitis (PBC), primary sclerosing cholangitis (PSC) and autoimmune hepatitis (AIH). Meaningful changes in disease epidemiology are reported, with increasing incidence and prevalence of AIH and PSC in Europe, and rising prevalence of PBC across Europe, North America and the Asia-Pacific region. However, there appears to be very significant global variation with contemporary incidence rates of disease per 100 000 ranging from 0.84 to 2.75 for PBC, 0.1 to 4.39 for PSC and 0.4 to 2.39 for AIH. Prevalence corresponds, and per 100 000 estimates for PBC range from 1.91 to 40.2, for PSC between 0.78 and 31.7 and for AIH from 4.8 to 42.9. Population-based studies and multicentre observational cohort series provide improved understanding of the clinical course that patients experience, highlighting variations in presenting phenotypes geographically and temporally. Collectively, while autoimmune liver diseases are rare, the clinical burden is disproportionately high relative to population incidence and prevalence. Age, sex and race also impact clinical outcomes, and patient morbidity and mortality are reflected by high need for gastroenterology, hepatology and organ transplant services.

Population-based study of autoimmune hepatitis and primary biliary cholangitis in Germany: rising prevalences based on ICD codes, yet deficits in medical treatment

2020 ◽  
Vol 58 (05) ◽  
pp. 431-438 ◽  
Author(s):  
Marcial Sebode ◽  
Angela Kloppenburg ◽  
Annette Aigner ◽  
Ansgar W. Lohse ◽  
Christoph Schramm ◽  
...  
Keyword(s):  
Autoimmune Hepatitis ◽  
Liver Diseases ◽  
Unmet Need ◽  
Real Life ◽  
Population Based ◽  
Medical Community ◽  
Primary Biliary Cholangitis ◽  
Autoimmune Liver Diseases ◽  
Background Population ◽  
Icd Codes

Abstract Background Population-based data on the prevalence of and real-life treatment for the autoimmune liver diseases (AILD), primary biliary cholangitis (PBC) and autoimmune hepatitis (AIH), are scarce, and such knowledge may help to improve patient care. Methods Data of 8.1 million individuals having health insurance with the “Techniker Krankenkasse,” the largest German health insurer, were analyzed with regard to the prevalence of and prescribed medication for AILD in Germany from 2011 until 2014. Patients with viral hepatitis B infection (HBV) and alcoholic liver cirrhosis (ALC) served as control groups. Case definition was based on ICD codes. Results The prevalences of PBC and AIH were 36.9/100 000 inhabitants (95 % CI: 35.6–38.2) and 23.0/100 000 inhabitants (95 % CI: 22.0–24.0) in 2014, respectively. The prevalences of AILD increased from 2011 to 2014 (for PBC by 31 % and for AIH by 29 %), with the largest increase for male patients with PBC. In contrast, the prevalence of HBV declined while that of ALC remained stable. The analysis of prescribed real-life treatment revealed considerable deviations from standard treatment recommendations. Specifically, in older patients with PBC or AIH, undertreatment was common. Conclusion The prevalence of PBC and AIH based on ICD codes is increasing in Germany. The analysis of real-life treatment in this large and population-based cohort points to the unmet need to improve the implementation of treatment guidelines for autoimmune liver diseases in the broader medical community.

Autoimmune Liver Diseases: Primary Biliary Cholangitis

2016 ◽  
pp. 251-287
Author(s):  
Ahmad H. Ali ◽  
Elizabeth J. Carey ◽  
Keith D. Lindor
Keyword(s):  
Liver Diseases ◽  
Primary Biliary Cholangitis ◽  
Autoimmune Liver Diseases

scholarly journals Antigen presentation, autoantibody production, and therapeutic targets in autoimmune liver disease

2020 ◽  
Vol 18 (1) ◽  
pp. 92-111 ◽  
Author(s):  
Andrea Kristina Horst ◽  
Kingsley Gideon Kumashie ◽  
Katrin Neumann ◽  
Linda Diehl ◽  
Gisa Tiegs
Keyword(s):  
Antigen Presentation ◽  
Immune Cells ◽  
Liver Diseases ◽  
Cell Activation ◽  
Tolerance Induction ◽  
Antigen Presenting Cells ◽  
Primary Biliary Cholangitis ◽  
Autoantibody Production ◽  
Autoimmune Liver Diseases ◽  
Antigen Presenting

AbstractThe liver is an important immunological organ that controls systemic tolerance. The liver harbors professional and unconventional antigen-presenting cells that are crucial for tolerance induction and maintenance. Orchestrating the immune response in homeostasis depends on a healthy and well-toned immunological liver microenvironment, which is maintained by the crosstalk of liver-resident antigen-presenting cells and intrahepatic and liver-infiltrating leukocytes. In response to pathogens or autoantigens, tolerance is disrupted by unknown mechanisms. Intrahepatic parenchymal and nonparenchymal cells exhibit unique antigen-presenting properties. The presentation of microbial and endogenous lipid-, metabolite- and peptide-derived antigens from the gut via conventional and nonconventional mechanisms can educate intrahepatic immune cells and elicit effector responses or tolerance. Perturbation of this balance results in autoimmune liver diseases, such as autoimmune hepatitis, primary biliary cholangitis, and primary sclerosing cholangitis. Although the exact etiologies of these autoimmune liver diseases are unknown, it is thought that the disruption of tolerance towards self-antigens and microbial metabolites and lipids, as well as alterations in bile acid composition, may result in changes in effector cell activation and polarization and may reduce or impair protective anti-inflammatory regulatory T and B cell responses. Additionally, the canonical and noncanonical transmission of antigens and antigen:MHC complexes via trogocytosis or extracellular vesicles between different (non) immune cells in the liver may play a role in the induction of hepatic inflammation and tolerance. Here, we summarize emerging aspects of antigen presentation, autoantibody production, and the application of novel therapeutic approaches in the characterization and treatment of autoimmune liver diseases.

scholarly journals Vitamin-D Receptor-Gene Polymorphisms Affect Quality of Life in Patients with Autoimmune Liver Diseases

Nutrients ◽  
2020 ◽  
Vol 12 (8) ◽  
pp. 2244 ◽  
Author(s):  
Agnieszka Kempinska-Podhorodecka ◽  
Monika Adamowicz ◽  
Mateusz Chmielarz ◽  
Maciej K. Janik ◽  
Piotr Milkiewicz ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Vitamin D ◽  
Vitamin D Receptor ◽  
Liver Diseases ◽  
Short Form ◽  
Receptor Gene ◽  
Primary Biliary Cholangitis ◽  
Vdr Gene ◽  
Autoimmune Liver Diseases

Vitamin D deficiency has been associated with depressive symptoms and reduced physical functioning. The aim of the study was to characterize the relationship between polymorphisms of the vitamin D receptor (VDR) gene and the quality of life in patients with autoimmune hepatitis (AIH) and primary biliary cholangitis (PBC). Three polymorphisms of the VDR gene (TaqI-rs731236, BsmI-rs1544410, and ApaI-rs7975232) were analyzed in patients with AIH (n = 142) and PBC (n = 230) and in healthy individuals (n = 376). Patient quality of life was assessed by validated questionnaires such as Medical Outcomes Study Short-Form 36 (SF-36), State Trait Anxiety Inventory (STAI), Modified Fatigue-Impact Scale (MFIS), Patient-Health Questionnaire 9 (PHQ-9), and PBC-40. The TaqI C and ApaI A alleles are risk alleles in both AIH and PBC, and a significant dominance of the A allele in BsmI was observed in AIH patients. In terms of quality of life, the presence of the CC or CT TaqI genotype was associated with emotional reactions, including the fatigue and the cognitive skills of patients with PBC, whereas in the group of AIH patients, homozygotes CC of TaqI, AA of BsmI, and AA of ApaI had worse physical, social, emotional, and mental function. The genetic variations of VDR gene can influence individual susceptibility to develop chronic autoimmune liver diseases such as AIH and PBC and affect quality of life.

Аутоиммунный гепатит на фоне описторхозной инвазии (клинический случай)

2020 ◽  
pp. 68-73
Keyword(s):  
Differential Diagnosis ◽  
Liver Cirrhosis ◽  
Early Diagnosis ◽  
Autoimmune Diseases ◽  
Disease Progression ◽  
Liver Diseases ◽  
Autoimmune Liver Diseases

The article describes the case of liver cirrhosis of autoimmune origin on the background of opisthorchiasis. The relevancy of the problem is justified by complexity of early diagnosis of autoimmune liver diseases on the background of opisthorchiasis, lack of data on the characteristics of the course of the combined pathology, and probable comorbidity of these diseases. These and many other questions still need to be addressed in future researches. The article shows the difficulty of differential diagnosis of autoimmune diseases of the liver and hepatitis of parasitic etiology, which leads to untimely, inadequate treatment and disease progression.

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