Background:
Primary sclerosing cholangitis is a rare chronic liver disease. It affects the bile ducts of the
liver. It can result in bile duct infections, cirrhosis, cancer, and end stage liver disease. Researchers want to learn more about this disease.
Objective:
To understand the biological causes of primary sclerosing cholangitis.
Eligibility:
Adults age 18 and older who have primary sclerosing cholangitis.
Design:
Participants will be screened with a medical history, physical exam, and blood tests.
Participants will give blood, saliva, urine, and stool samples. They will have nasal swabs. They will complete surveys.
Participants will get an intravenous (IV) catheter. A plastic tube is inserted into an arm vein.
Participants will have a colonoscopy. A tube with a video camera at the end is inserted into the rectum.
Participants will have an upper endoscopy. A scope with a light and camera at its tip is used to look inside the upper digestive tract.
Participants will have a liver biopsy, entering through the chest wall or a neck vein. Blood is drawn from a blood vessel that carries blood to the liver. A liver tissue sample is taken.
Participants will have magnetic resonance imaging or spectroscopy. They will get a contrast agent through an IV.
Participants may have an optional bone marrow aspiration. A large needle is inserted into the hip to withdraw marrow.
Participants will have a liver ultrasound.
Participants will complete a 3-day food diary. They will have a nutrition assessment.
Participants may give contact details for people who live with them, to also take part in this study.
Participation will last for 12 months.
Stated willingness to comply with all study procedures and availability for the duration of the study
Male or nonpregnant female, greater than or equal to 18 years of age
Evidence of PSC established by biochemical testing and either MRCP or ERCP. Participant must have evidence of large duct disease on imaging.
Agreement to adhere to Lifestyle Considerations throughout study duration.
Pregnant or lactating women or females of child-bearing age not taking measures to prevent pregnancy during the period of study.
History of clinical, serologic, or histopathologic evidence supporting etiologies of chronic liver disease other than PSC
History of liver transplantation
Diagnosis consistent with secondary sclerosing cholangitis (cholelithiasis, bile duct strictures secondary to ischemia, HIV cholangiopathy, etc.).