Primary Sclerosing Cholangitis
Conditions
Brief summary
Primary sclerosing cholangitis (PSC) is a chronic progressive biliary disease that affects approximately 1200 patients in the Netherlands and around 80,000 in the Western world. It is often accompanied by ulcerative colitis (UC) or Crohn's disease affecting the large bowel. The cause of PSC is unknown, there is no medical therapy available that has proven to halt disease progression and the median time until death or liver transplantation is 13-21 years. Diagnosis is made by magnetic resonance cholangiography (MRC), or in the case of so called small duct disease by liver biopsy. Due to the heterogeneous disease course and the relatively low clinical event rate of 5% per year it is difficult to predict prognosis of individual patients or to recommend any surveillance strategy for malignancies. Also, the lack of surrogate endpoints impedes performing clinical research. Recently, two new post-processing tools have been developed to characterize and quantify abnormalities in the biliary tree as well as excretory function captured by MRC. These tools called MRCP+ (quantitative magnetic resonance cholangiopancreatography +) and LiverMultiscan (LMS) hold the prospect of adequately depicting and quantifying lesions of the biliary tree as well as capturing functional derailment. However, several features must be tested before the utility of this tools in clinical patient care can be concluded. Therefore, the aim of this study is to investigate the utility of these novel techniques in monitoring disease activity by performing consecutive annual MRI's.
Interventions
DEVICELiver Multiscan sequences
Additional Liver Multiscan sequences will be performed after the MRI liver with MRCP is performed, taking approximately 15 minutes.
DEVICELiver Multiscan analysis
Post processing tool (Software) for determining the corrected T1 time after the additional LMS sequences at baseline are performed. This cT1 reflects the activity of inflammation/fibrosis of the liver. Patient involvement is not necessary during this procedure.
DEVICEMRCP+
Post processing tool (Software) for quantifying MRCP images after the MRCP from follow up is performed. Patient involvement is not necessary during this procedure.
Sponsors
Perspectum
Academisch Medisch Centrum - Universiteit van Amsterdam (AMC-UvA)
Study design
Allocation
NA
Intervention model
SINGLE_GROUP
Primary purpose
DIAGNOSTIC
Masking
NONE
Eligibility
Sex/Gender
ALL
Age
18 Years to No maximum
Healthy volunteers
No
Inclusion criteria
* Established PSC diagnosis according to the IPSCSG definitions * Age ≥ 18 * Able to give informed consent
Exclusion criteria
* Post LTx * Known allergy for MRI contrast agents, implants non-compatible with MRI or extreme claustrophobia causing discontinuation of MRI studies.
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Delta of cT1 in patients with PSC during follow-up | 1st MRI = baseline = week 0; 2nd MRI = year 1 = week 52; 3rd MRI = year 2 = week 104; 4th MRI = year 3 = week 156; 5th MRI = year 4 = week 208; 6th MRI = year 5 = week 260. | The delta of cT1 (in ms), measured by LiverMultiscan, which will be assessed by performing paired t-tests. |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Variance of the delta of cT1 and MRCP+ metrics in stable patients | 1st MRI = baseline = week 0; 2nd MRI = year 1 = week 52; 3rd MRI = year 2 = week 104; 4th MRI = year 3 = week 156; 5th MRI = year 4 = week 208; 6th MRI = year 5 = week 260. | Variance of the delta in cT1 and MRCP+ metrics from baseline to year 1 in clinically and biochemically stable patients |
| Difference in cT1 in patients with or without endoscopic intervention | 1st MRI = baseline = week 0; 2nd MRI = year 1 = week 52; 3rd MRI = year 2 = week 104; 4th MRI = year 3 = week 156; 5th MRI = year 4 = week 208; 6th MRI = year 5 = week 260. | Difference in mean of cT1 values in patients who needed ERCP with treatment of dominant stricture(s) in the year following LMS measurement versus those who did not need an intervention |
| Variance in unstable patients | 1st MRI = baseline = week 0; 2nd MRI = year 1 = week 52; 3rd MRI = year 2 = week 104; 4th MRI = year 3 = week 156; 5th MRI = year 4 = week 208; 6th MRI = year 5 = week 260. | Variance of the delta of cT1 and MRCP+ metrics in sequential scans in biochemically and/or clinically deteriorating patients. |
| Assesment of th mean, median and range of cT1 and MRCP+ metrics in stable PSC patients | 1st MRI = baseline = week 0; 2nd MRI = year 1 = week 52; | Mean, median and range of cT1 and MRCP+ metrics in clinically and biochemically stable patients with PSC during the period of 1 year to establish general statistics as these are unknown at current writing |
| Correlation of cT1 and MRCP+ metrics and development of dominant strictures | 1st MRI = baseline = week 0; 2nd MRI = year 1 = week 52; 3rd MRI = year 2 = week 104; 4th MRI = year 3 = week 156; 5th MRI = year 4 = week 208; 6th MRI = year 5 = week 260. | Correlation between cT1 and MRCP+ metrics and development of dominant strictures |
| Correlation of cT1 and MRCP+ metrics and incidence of CCA | 1st MRI = baseline = week 0; 2nd MRI = year 1 = week 52; 3rd MRI = year 2 = week 104; 4th MRI = year 3 = week 156; 5th MRI = year 4 = week 208; 6th MRI = year 5 = week 260. | Correlation between cT1 and MRCP+ metrics and incidence of CCA |
| Correlation of cT1 and MRCP+ metrics with fibroscan and MELD | 1st MRI = baseline = week 0; 2nd MRI = year 1 = week 52; 3rd MRI = year 2 = week 104; 4th MRI = year 3 = week 156; 5th MRI = year 4 = week 208; 6th MRI = year 5 = week 260. | Correlation between cT1 and MRCP+ metrics with Fibroscan and MELD-score |
Contacts
Primary ContactTim E Middelburg, MSC
Outcome results
None listed