Cazzagon, Nora (2018) The prognostic role of magnetic resonance imaging in Primary Sclerosing Cholangitis. [Ph.D. thesis]
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Abstract (italian or english)
Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease of unknown aetiology characterized by inflammation and obliterative fibrosis of the biliary tree. Although the course is highly variable, PSC is often progressive, leading to biliary cirrhosis and its complications. Overall, PSC is a severe disease and median transplant-free survival ranges from 13 years in patients seen at tertiary referral centres to 20 years in a population-based cohort. Liver transplantation is the only life-extending therapeutic intervention for patients with end-stage liver disease. No medical therapy has proven to be effective to halt disease progression. The clinical course of the disease is highly variable among different patients. Identification of prognostic factors is essential for tailoring the follow-up strategies and/or testing new therapeutic modalities in subgroups of PSC patients with poor prognosis. Magnetic resonance cholangiography is recommended as the first diagnostic modality in patients with suspected PSC and its potential role in assessing prognosis is justified by its non-invasive nature, its reproducibility and its adequate performance in assessing biliary alterations in PSC that resulted comparable to that of endoscopic cholangiography. The aim of this thesis was to assess the prognostic value of magnetic resonance imaging in these patients. I thus assessed the prognostic value of two MR risk scores in predicting the risk of adverse outcomes and I evaluated whether the use in combination of MR risk score with liver stiffness may improve risk stratification of these patients. I evaluated the radiological and clinical course of PSC patients with intrahepatic bile ducts cystic dilatations found at magnetic resonance cholangiography and I assessed the prognostic value of this finding. Finally, I assessed the existence of magnetic resonance cholangiography and clinical criteria of improvement after endoscopic treatment for severe strictures. The results of this research demonstrated that two simple MR risk score were associated to the risk of adverse outcomes in PSC patients and that the use in combination of MR risk score without gadolinium-based contrast agent injection with liver stiffness was able to improve risk stratification in these patients. I reported on a subgroup of patients with cystic dilatations of intrahepatic bile ducts and I documented that these patients were characterized by a younger age at PSC diagnosis, a rapid radiological and clinical evolution, and a significantly lower median transplant free survival from PSC diagnosis compared with PSC patients without cystic dilatations. Finally, I documented that some magnetic resonance cholangiography features together with biochemical features, may contribute to identify PSC patients likely to improve after endoscopic treatment for severe strictures of extrahepatic bile ducts.
In conclusion, I here provided some evidences in favour of the use of magnetic resonance imaging for prognostic, other than diagnostic, purposes in PSC.
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