Sex: All
Minimum Age: 18
Healthy Volunteers: Accepts Healthy Volunteers
INCLUSION CRITERIA:
In order to be eligible to participate in this study, an individual must meet all of the following criteria:
- 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.
INCLUSION CRITERIA:
In order to be eligible to participate in this study, an individual must meet all of the following criteria:
- Male or female greater than or equal to 18 years of age
- Any individual who is either a parent, sibling or child of a PSC patient enrolled to the study, or an unrelated person who has been living with the patient for at least 3 consecutive months before study enrollment
EXCLUSION CRITERIA:
An individual who meets any of the following criteria will be excluded from participation in this study:
- 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.).
- Current or past clinical evidence of decompensated liver disease (e.g. ascites, bleeding esophageal varices, spontaneous bacterial peritonitis, encephalopathy, etc.).
- History of liver or bile duct lesions concerning for malignancy.
- Ca-19-9 >130 U/microL
- Alpha-fetoprotein level greater than 200 ng/microL.
- Patients with active bacterial, viral, or fungal, systemic or localized infection.
- Unwillingness to refrain from ingesting probiotics during study.
- History of systemic disease not related to PSC that is poorly controlled or associated with declining functional status. Examples include but are not limited to: poorly controlled diabetes mellitus, chronic renal failure with eGFR is <60 microl/min/1.73m^2, chronic
- symptomatic heart failure or severe COPD.
- Patients with history of malignancy other than superficial skin cancer (BCC/SCC) in the last 3 years prior to enrolment.
- History of portal vein thrombosis
- Patients with severe allergic reactions to iodine or other contrast, which cannot be controlled by premedication with antihistamines or steroids.
- History of gastric and/or proximal small bowel surgery including bariatric surgery such as Roux-en-Y gastric bypass
- Contraindication to monitored anesthesia care and/or medications that are commonly used for conscious sedation during GI Endoscopy
- Use of anti-coagulant and anti-platelet agents excluding aspirin and NSAIDs
- Contraindications to completing MRCP or MRI
- Absolute neutrophil count below 1000/mm^3
- Hemoglobin level below 10.0 g/dl
- Platelet count lower than 50,000/mm^3.
- INR greater than or equal to 1.5, PTT greater thna or equal to 1.3 times control and/or any known history of disease associated with
- increased bleeding diathesis.
- Inability to provide informed consent
CONTROLS:
EXCLUSION CRITERIA:
An individual who meets any of the following criteria will be excluded from participation in this study:
- History suggestive of PSC
- History of chronic liver disease (except for steatosis)
- History of malignancy other than superficial skin cancer (BCC/SCC) in the last 3 years prior to enrollment.
- History of Inflammatory Bowel Disease
- Antibiotic use within the last 6 weeks
- Pregnancy
- Inability to provide informed consent