Clinical application of annual Liver Multiscan and MRCP+ in Primary Sclerosing Cholangitis:

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 MRCP+ and Liver
Multiscan analysis on in standard-care annual MRI*s .

Main:
The main objective is to explore the potential utility of MRCP+ and LMS in
monitoring disease activity and early detection of worsening disease requiring
endoscopic intervention.

Secondary Objective(s):
Assessment of mean, median, range and variance of MRCP+ metrics and cT1 in PSC
patients at baseline and follow-up.
Finding clinically relevant deltas of cT1 (ms) and MRCP+ metrics in patients
with both clinically and/or biochemically stable and worsening disease
Assessment of the correlation between changes in LMS metrics with changes in
fibroscan and MELD-score.
Assessment of the correlation between (segmental) cT1 and MRCP+ metrics with
the development of dominant strictures
Assessment of the correlation between cT1 values and the incidence of CCA in
follow up. Assessment of the value of annual follow up by MRI with contrast,
MRCP+ and LMS in the detection of cholangiocarcinoma and gallbladder carcinoma.

Prospective, single center study.

There is little burden or risk associated with this study as it only requires a
few extra sequences in addition to annual MRI that is performed routinely in
standard-care settings.