Early Identification of Myocardial Impairment in PBC

Study Overview
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Brief Summary
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Primary biliary cholangitis (PBC) is a chronic inflammatory liver disease leading to cirrhosis. Researches reported patients with PBC may involve abnormalities on skeleton, thyroid and exocrine glands. However, whether this autoimmune disease would cause cardiac impairment is scarcely investigated. Cardiovascular Magnetic Resonance(CMR) is recently developed as a reliable modality to evaluate the cardiac tissue characteristics and functions. This study aims to investigate the cardiac status in PBC patients based on CMR.

Detailed Description
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Primary biliary cholangitis (PBC) is a progressive and uncommon inflammatory autoimmune cholesteric liver disease，which will contribute to cirrhosis. Symptoms and course of primary biliary cholangitis can be diverse, wherefore the targets of the current treatment are focused on the prevention of end-stage liver disease. Researches reported patients with PBC may involve abnormalities on skeleton, thyroid and exocrine glands. However, whether this autoimmune disease would cause cardiac impairment is scarcely investigated. From our clinical practice, the cardiac structural abnormal can be found in certain patients with PBC detected by cardiovascular magnet resonance (CMR). CMR is the primary and emerging imaging modality for myocardial tissue characterisation, and it is recommended as a gold standard for functional imaging and assessment. This single-center, prospective observational study plans to identify the type and the severity of cardiac changes in PBC.

Official Title
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EARLY Identification of MYOcardial Impairment in Primary Biliary Cholangitis

Conditions
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Primary Biliary Cholangitis, Cardiovascular Abnormalities

Intervention / Treatment
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* Diagnostic Test: CMR examination


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria for PBC group: Age between 18-80 years old. Definite primary biliary cirrhosis diagnosis which is consistent with European Association for the Study of the Liver (EASL) [Clinical Practice Guidelines: The diagnosis and management of patients with primary biliary cholangitis (2017)]. The following three diagnostic factors, at least meet two: History of elevated alkaline phosphatase (ALP) levels; Liver biopsy consistent with PBC; Positive antimitochondrial antibodies (AMA) or specific antinuclear antibodies. Providing written informed consent Exclusion Criteria: History or presence of other concomitant liver disease including: Viral hepatitis; Inherited metabolic liver disease; Drug-induced liver injury; Other systemic disease inducing liver change. Subjects with known ischemic/non-ischemic cardiomyopathy. Subjects with standard metallic contraindications to CMR (i.e., estimated glomerular filtration rate < 30 ml/min/1.73 m2, New York Heart Association functional capacity class IV) Inclusion Criteria for Control group: Absence of known systemic diseases Normal examinations in CMR/Echo/ECG Age between 18-80 years old. Providing written informed consent Exclusion Criteria: Subjects with known heart disease including: Documented coronary artery disease; Ischemia/non-ischemia cardiomyopathy; Other systemic disease inducing heart change. Subjects with known liver disease including: Viral hepatitis; Inherited metabolic liver disease; Drug-induced liver injury; Other systemic disease inducing liver change. Subjects with standard metallic contraindications to CMR

Ages Eligible for Study
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Minimum Age: 18 Years
Maximum Age: 80 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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Accepts Healthy Volunteers

Study Plan
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How is the study designed?
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Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| PBC group<br>Patients have a definite PBC diagnosis. | Diagnostic Test: CMR examination<br>* After recruiting participants and collecting the baseline information, a CMR scan and a post-processed imaging procedure will be carried on in order to detect the cardiac impairment.<br>|
| Control group<br>The healthy volunteers or patients in Renji Hospital whose medical examinations show no systemic disease, and the CMR examinations are normal. | Diagnostic Test: CMR examination<br>* After recruiting participants and collecting the baseline information, a CMR scan and a post-processed imaging procedure will be carried on in order to detect the cardiac impairment.<br>|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| The incidence of cardiac involvement | Assessments base on the cardiac structure, function and tissue characterization | through study completion, an average of 14 months | 
| Composite endpoint of quantitative assessment in cardiac injury | Compose of extracellular volume(%), T1 value(ms) and T2 value(ms). | within 2 days of CMR scan | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Composite endpoint of liver condition | Compose of the alanine aminotransferase (ALT), Aspartate aminotransferase (AST) ,alkaline phosphatase (AKP) and bilirubin indicating the liver condition and prognosis. | within 2 days of CMR scan | 
| Composite endpoint of cardiac function | Compose of CRP (hs-CRP), troponin I (TnI) and hematocrit indicating the cardiac function | within 2 days of CMR scan | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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Primary Biliary Cholangitis, Cardiac impairment, Cardiovascular Magnetic Resonance