Early Identification of Myocardial Impairment in PBC

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.

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 characterization, and it is recommended as a gold standard for functional imaging and assessment. This three-center, multi-modality, prospective observational study plans to identify the type and the severity of cardiac changes in PBC.

Eligibility Criteria:
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:

  1. History of elevated alkaline phosphatase (ALP) levels;
  2. Liver biopsy consistent with PBC;
  3. Positive antimitochondrial antibodies (AMA) or specific antinuclear antibodies;
* Providing written informed consent

Exclusion Criteria:

* History or presence of other concomitant liver disease including:

  1. cirrhosis or viral hepatitis;
  2. Inherited metabolic liver disease;
  3. Drug-induced liver injury;
  4. Other systemic disease inducing liver change.
* Subjects with life expectancy \< 6 months.
* Subjects with known ischemic/non-ischemic cardiomyopathy or abnormal in cardiac-related examinations.
* 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:

  1. Documented coronary artery disease;
  2. Ischemia/non-ischemia cardiomyopathy;
  3. Other systemic disease inducing heart change.
* Subjects with known liver disease including:

  1. Viral hepatitis;
  2. Inherited metabolic liver disease;
  3. Drug-induced liver injury;
  4. Other systemic disease inducing liver change.
* Subjects with standard metallic contraindications to CMR