• Elasticity Imaging Techniques/methods
• Humans
• Liver Cirrhosis/diagnostic imaging
• Liver Cirrhosis/etiology
• Liver Cirrhosis/pathology
• Cholestasis/complications
• Cholestasis/diagnostic imaging
• Sensitivity and Specificity
• Liver Cirrhosis, Biliary/diagnostic imaging
• Liver Cirrhosis, Biliary/complications
• Liver Cirrhosis, Biliary/pathology
• Cholangitis, Sclerosing/diagnostic imaging
• Cholangitis, Sclerosing/complications
• Cholangitis, Sclerosing/pathology

• Chronic liver disease
• Guidelines
• Liver fibrosis
• Noninvasive test

• Humans
• Liver Cirrhosis/diagnosis
• Liver Cirrhosis/complications
• Chronic Disease
• Elasticity Imaging Techniques
• Biomarkers
• Liver Diseases/diagnosis
• Liver Diseases/complications
• Liver Diseases/pathology

• Autoimmune disease
• Liver fibrosis
• Noninvasive test
• Transient elastography

• Humans
• Elasticity Imaging Techniques
• Liver Cirrhosis/diagnosis
• Liver Cirrhosis/complications
• Liver Cirrhosis/pathology
• Vibration
• Hepatitis, Autoimmune/complications
• Hepatitis, Autoimmune/diagnosis
• Cholangitis, Sclerosing/complications
• Cholangitis, Sclerosing/diagnosis
• Liver Cirrhosis, Biliary/diagnosis
• Liver Cirrhosis, Biliary/complications
• Liver Cirrhosis, Biliary/pathology
• ROC Curve
• Liver/pathology
• Liver/diagnostic imaging
• Autoimmune Diseases/complications
• Autoimmune Diseases/diagnosis

• RS-2022-00166674 National Research Foundation of Korea

• diagnosis
• gastric varix
• idiopathic portal hypertension
• portal hypertension
• transient elastography

• Autoimmune liver disease
• Liver fibrosis
• Liver stiffness
• Noninvasive diagnosis
• Serum parameter
• Transient elastography

• Humans
• Elasticity Imaging Techniques/methods
• Liver Cirrhosis/diagnosis
• Liver Cirrhosis/diagnostic imaging
• Fibrosis
• Hepatitis, Autoimmune/complications
• Hepatitis, Autoimmune/diagnostic imaging
• ROC Curve
• Non-alcoholic Fatty Liver Disease/pathology
• Aspartate Aminotransferases
• Liver/diagnostic imaging
• Liver/pathology

Primary biliary cholangitis (PBC) is a chronic progressive cholestatic disease characterized by destruction of small- and medium-sized intrahepatic bile ducts. It is no longer a rare disease, since many new asymptomatic cases are incidentally identified. Liver biopsy is diagnostically critical but not always feasible or practical to be performed. Many potential, noninvasive, markers have been proposed to replace liver biopsy and further provide the assessment of disease severity and ultimate prognosis. In this review, we evaluated serum biomarkers proposed for diagnosis, extent of fibrosis, disease prognosis and attempts for early prediction of treatment response. Older biochemical and immunological markers are presented along with recent reports including the role of microRNAs and promising results based on proteomics and metabolomics.

• autoantibodies
• microRNAs
• primary biliary cholangitis
• proteomics
• treatment response

The GLOBAL Primary Biliary Cholangitis (PBC) Study Group and United Kingdom‐PBC (UK‐PBC) Consortium have demonstrated that dichotomous response criteria are not as accurate as continuous equations at predicting mortality or liver transplantation in PBC. The aim of this analysis was to assess the clinical utility of the GLOBE and UK‐PBC risk scores using data from POISE, a phase 3 trial investigating obeticholic acid (OCA) in patients with PBC. Data (N = 216) at baseline and month 12 were used to calculate the GLOBE and UK‐PBC risk scores to assess the projected change in risk with OCA versus placebo. Additionally, the benefit of OCA was assessed in patients not meeting the POISE primary endpoint. Both the GLOBE and UK‐PBC risk scores predicted a significant reduction in long‐term risk of death and liver transplantation after OCA treatment (P < 0.0001). The differences in the relative risk reduction from baseline in the 10‐year event risk after 1 year for OCA 10 mg versus placebo was 26% (GLOBE) and 37% (UK‐PBC). The scores also predicted a significantly decreased risk in patients treated with OCA who did not meet POISE response criteria after 1 year of treatment compared to an increased risk with placebo (P < 0.0001). Conclusion: This analysis demonstrates the use of the GLOBE and UK‐PBC risk scores to assess risk reduction of a cohort treated with OCA. While validation of this risk reduction in studies with clinical outcomes is needed, this study highlights the potential use of these scores in individualizing risk prediction in PBC both in clinical practice and therapeutic trials. (Hepatology Communications 2018;2:683‐692)

• compensated advanced chronic liver disease
• elastography
• hepatic venous pressure gradient
• ultrasound

• Chronic liver diseases
• Elastography
• Non-invasive methods
• Non-invasive serum markers
• Stiffness

• Biomarkers/analysis
• Biopsy
• Disease Progression
• Elasticity Imaging Techniques/methods
• Humans
• Liver Cirrhosis/diagnosis
• Liver Cirrhosis/pathology
• Liver Cirrhosis/therapy
• Predictive Value of Tests
• Prognosis
• Treatment Outcome

• Autoimmune biliary disease
• Bile duct
• Hepatobiliary
• Primary biliary cirrhosis
• Sclerosing cholangitis

• APRI
• Autoimmune Hepatitis
• FibroQ
• NAFLD Fiibrosis Score
• Transient Elastography

Patients with primary sclerosing cholangitis (PSC) develop progressive liver fibrosis and end-stage liver disease. Non-invasive and widely available parameters are urgently needed to assess disease stage and the risk of clinical progression. Transient elastography (TE) has been reported to predict fibrosis stage and disease progression. However, these results have not been confirmed in an independent cohort and comparison of TE measurement to other non-invasive means is missing.

In a retrospective study we collected data from consecutive PSC patients receiving TE measurements from 2006 to 2014 (n = 139). Data from 62 patients who also underwent a liver biopsy were used to assess the performance of TE and spleen length (SL) measurement for the staging of liver fibrosis. Follow-up data from this cohort (n = 130, Hamburg) and another independent cohort (n = 80, Paris) was used to compare TE and SL as predictors of clinical outcome applying Harrel’s C calculations.

TE measurement had a very good performance for the diagnosis and exclusion of higher fibrosis stages (≥F3: AUROC 0.95) and an excellent performance for the diagnosis and exclusion of cirrhosis (F4 vs. < F4: AUROC 0.98). Single-point TE measurement had very similar predictive power for patient outcome as previously published. In a combined cohort of PSC patients (n = 210), SL measurements had a similar performance as TE for the prediction of patient outcome (5 x cross-validated Harrel’s C 0.76 and 0.72 for SL and TE, respectively).

Baseline TE measurement has an excellent performance to diagnose higher fibrosis stages in PSC. Baseline measurements of SL and TE have similar usefulness as predictive markers for disease progression in patients with PSC.

Primary biliary cirrhosis (PBC), primary sclerosing cholangitis (PSC) and autoimmune hepatitis (AIH) constitute the classic autoimmune liver diseases (AILDs). While AIH target the hepatocytes, in PBC and PSC the targets of the autoimmune attack are the biliary epithelial cells. Persistent liver injury, associated with chronic AILD, leads to un-resolving inflammation, cell proliferation and the deposition of extracellular matrix proteins by hepatic stellate cells and portal myofibroblasts. Liver cirrhosis, and the resultant loss of normal liver function, inevitably ensues. Patients with cirrhosis have higher risks or morbidity and mortality, and that in the decompensated phase, complications of portal hypertension and/or liver dysfunction lead to rapid deterioration. Accurate diagnosis and monitoring of cirrhosis is, therefore of upmost importance. Liver biopsy is currently the gold standard technique, but highly promising non-invasive methodology is under development. Liver transplantation (LT) is an effective therapeutic option for the management of end-stage liver disease secondary to AIH, PBC and PSC. LT is indicated for AILD patients who have progressed to end-stage chronic liver disease or developed intractable symptoms or hepatic malignancy; in addition, LT may also be indicated for patients presenting with acute liver disease due to AIH who do not respond to steroids.

• Hepatic fibrosis
• Cirrhosis
• Myofibroblasts
• Primary biliary cirrhosis
• Primary sclerosing cholangitis
• Autoimmune hepatitis
• Liver transplantation

Primary biliary cirrhosis (PBC) is a chronic non-suppurative destructive intrahepatic cholangitis leading to cirrhosis after a protractive non cirrhotic stage. The etiology and pathogenesis are largely unknown and autoimmne mechanisms have been implicated to explain the pathological lesions. Many epitopes and autoantigens have been reported as crucial in the pathophysiology of the disease and T and B cells abnormalities have been described, the exact pathways leading to the destruction of small intrahepatic ductules are mostly speculative. In this review we examined the various epidemiologal and geoepidemiological data as well as the complex pathogenetic aspects of this disease, focusing on recent in vivo and in vitro studies in this field. Initiation and progression of PBC is believed to be a multifactorial process with strong infuences from the patient’s genetic background and by various environmental factors. The role of innate and adaptive immunity, including cytokines, chemokines, macrophages and the involvement of apoptosis and reactive oxygen species are outlined in detailed. The current pathogenetic aspects are presented and a novel pathogenetic theory unifying the accumulated clinical information with in vitro and in vivo data is formulated. A review of clinical manifestations and immunological and pathological diagnosis was presented. Treatment modalities, including the multiple mechanisms of action of ursodeoxycholate were finally discussed.

• Primary biliary cirrhosis
• Innate immunity
• Adaptive immunity
• Ursodeoxycholate
• Chemokines
• Macrophages
• Cytokines

AIM: To evaluate the value of fibroscan (FS) in hepatic fibrosis stage assessment in patients with chronic viral hepatitis, and to examine whether its accuracy is affected by etiology.

METHODS: English and Chinese articles related to assessment of hepatic fibrosis stage by fibroscan in Wanfang, Chinese Journal Full-Text Database (CJFD), Chinese Biomedical Literature Database (CBM), PubMed, Cochrane library and EMBASE database were strictly screened and evaluated. Data of enrolled articles were analyzed using Meta-disc1.4 and Stata12.0 software.

RESULTS: A total of 28 English and Chinese articles were included. The pooled sensitivity, specificity, diagnostic odds ratio and the area under curve (AUC) of summary receiver operating characteristic (SROC) curve for significant fibrosis (F ≥ 2) and cirrhosis (F = 4) in patients with chronic viral hepatitis were 0.72 (0.70-0.73), 0.85 (0.83-0.87), 18.51 (13.28-25.80), 0.88 and 0.86 (0.84-0.88), 0.86 (0.85-0.87), 49.14 (30.53-79.09) and 0.94, respectively. There was no significant difference among the results of meta-analysis according to etiology.

CONCLUSION: Fibroscan has a high diagnostic accuracy for evaluating hepatic fibrosis stage, especially in patients with cirrhosis. The diagnostic accuracy of fibroscan is not affected by etiology.

• Fibroscan
• Hepatic fibrosis
• Meta-analysis
• Chronic hepatitis C
• Chronic hepatitis B

• ARFI imaging
• Autoimmune hepatitis
• Primary biliary cirrhosis
• Primary sclerosing cholangitis