Recruiting

Observational Study

Sponsor:

Children's Hospital Medical Center, Cincinnati

Code:

NCT03175471

Conditions

Autoimmune Liver Disease

Primary Sclerosing Cholangitis

Autoimmune Hepatitis

Eligibility Criteria

Sex: All

Age: 6 - 23

Healthy Volunteers: Not accepted

Study Details

Brief summary:

Autoimmune liver diseases (AILD), which include Primary Sclerosing Cholangitis (PSC) and Autoimmune Hepatitis (AIH) are a common etiological factors for chronic liver disease among adolescents. In all these conditions, autoimmune lymphocyte responses are thought to orchestrate inflammatory injury against hepatocytes (primarily in AIH) or cholangiocytes (in PSC). In this proposal we aim to evaluate the Magnetic Resonance Imaging (MRI) modalities; MR cholangiopancreatography (MRCP) and MR elastography (MREL), as non-invasive biomarkers to assess two primary pathophysiological processes of AILD: bile duct damage and liver fibrosis. In this cross-sectional study MRI based findings of bile duct injury and liver fibrosis will be correlated with both liver histology and circulating biomarkers of these disease processes.

Conditions

Autoimmune Liver Disease

Primary Sclerosing Cholangitis

Autoimmune Hepatitis

Study ID

NCT03175471

Start date

Jan 17, 2017

Status verified date

Aug, 2026

Completion date

Jan 30, 2030

Anticipated

Primary completion date

Jan 30, 2030

Anticipated

Eligibility Criteria

Eligibility Criteria

Sex: All

Age: 6 - 23

Healthy Volunteers: Not accepted

Inclusion Criteria:

1. Age 6-23 years old.
2. Established or suspected clinical diagnosis of AIH or PSC.

Exclusion Criteria:

1. History of liver transplantation.
2. Chronic Hepatitis B or untreated hepatitis C virus infection.
3. Pregnancy.
4. Absolute contraindication for MRI (e.g. pacemaker, metallic implants, claustrophobia).
5. Diagnosis of cystic fibrosis or biliary atresia
6. Diagnosis of cardiac hepatopathy.
7. Diagnosis of Wilson's disease, Alpha-1 Antitrypsin deficiency, or Glycogen storage disease.
8. Skin conditions which could be aggravated by MREL (i.e. Epidermolysis bullosa).

Study Design

Enrollment

115 participants

Anticipated

Interventions and Outcome Measures

Arms

Patients with autoimmune liver disease

Patients (6-23 y.o.) with established clinical diagnosis of AIH or suspected diagnosis of AIH based on elevated serum AST or ALT, elevated IgG level >1.1 ULN, elevated titer of autoantibodies, including ANA, SMA, LKM, LC-1 or SLA, which is consistent with the simplified criteria for the diagnosis of AIH in children will be enrolled.

Patients (6-23 y.o.) with established clinical diagnosis of PSC or Suspected diagnosis of PSC supported by abnormal cholangiogram (ERCP or MRCP) or elevated GGT>1.5 ULN and dilated bile ducts by liver ultrasound will be enrolled.

Primary outcome measure

  • MRI based outcomes [ Time Frame: 36 months ]
  • Liver histopathology based assessment of bile duct injury by ISHAK Score [ Time Frame: 36 months ]
  • Liver histopathology based assessment of bile duct injury by Ludwig score [ Time Frame: 36 months ]
  • Liver histopathology based assessment of liver fibrosis by Nakanuma score [ Time Frame: 36 months ]
  • Liver histopathology based assessment of liver fibrosis by Ishak score [ Time Frame: 36 months ]
  • Liver histopathology based assessment of cholangitis and hepatic activity [ Time Frame: 36 months ]
  • Serum based outcome [ Time Frame: 36 months ]
  • Enhanced Liver Fibrosis (ELF) score [ Time Frame: 36 months ]

Central Contacts and Locations

Central contacts

Alexander Miethke, MD

513-636-8948Alexander.Miethke@cchmc.org

Cyd Castro Rojas, PhD

513-517-0580Cyd.CastroRojas@cchmc.org

Locations

Cincinnati Childrens Hospital Medical Center

Recruiting

Cincinnati, Ohio, United States, 45229

Contacts

Alexander Miethke, MD

513-636-5581Alexander.Miethke@cchmc.org

Principal Investigator:

Alexander Miethke, MD

More Information

Sponsor

Children's Hospital Medical Center, Cincinnati

Last update posted

Aug 19, 2026

Last verified

Aug, 2026

Trial information was received from ClinicalTrials.gov and was last updated on 2026-09-10. This information was provided to ClinicalTrials.gov by Children's Hospital Medical Center, Cincinnati on 2026-08-19.