Primaarinen sklerosoiva kolangiitti : Epidemiologia, ennuste, liitännäissairaudet ja kuolleisuus

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Background: Primary Sclerosing Cholangitis (PSC) is a rare, chronic liver disease characterized by inflammation and fibrosis of the bile ducts, often leading to liver cirrhosis and, eventually, liver failure. There is an increased risk of malignancy, especially hepatobiliary malignancy. Despite significant morbidity, effective treatments remain limited, and early diagnosis is challenging. This study aims to analyse PSC epidemiology, prognosis and risk of malignancy and secondly, evaluate risk factors for cholangiocarcinoma (CCA), its prognosis, and epidemiology in a population-based cohorts. For PSC surveillance we compared three surveillance strategies—scheduled endoscopic retrograde cholangiography (ERC), annual magnetic resonance imaging (MRI)/ magnetic resonance cholangiopancreatography (MRCP surveillance), and on-demand ERC. Patients and Methods: We conducted a population-based cohort study in Finland, utilising data from national health registries and Helsinki University Hospital's (HUH) registries to analyse patients diagnosed with PSC in the HUH area from 1990-2015. All CCA patients from 1974 to 2018 were retrieved from the Finnish cancer registry. Patients from the HUH PSC registry were retrieved to calculate PSC as a risk factor for CCA. Other risk factors were calculated by linking the nationwide cohort with the nationwide patient registry (HILMO). The HUH PSC registry was used to obtain the patients for the HelPSCreen score study. We used 13 laboratory tests and the patient's demographics to develop a diagnostic screening tool for PSC. We gathered data from three different surveillance cohorts in a multinational study. To assess the different surveillance strategies, we used the composite endpoints, liver transplantation (LT), hepatobiliary malignancies, and liver-related mortality. Results: The incidence of PSC in Finland was found to align with reported rates in other Nordic countries. However, the point prevalence in 2015 was higher than previously reported in other countries, 31.7 per 100000 inhabitants. Patients with PSC exhibited a markedly higher risk of developing CCA, with 44% of CCA diagnoses occurring within the first year of PSC diagnosis. PSC was also identified as the most significant risk factor for CCA, with PSC patients developing CCA at a much younger age (mean age 51 years) than non-PSC CCA patients (mean age 73 years). Among surveillance strategies, scheduled ERC with brush cytology resulted in the lowest cumulative incidence of adverse clinical outcomes compared to annual MRI/MRCP and on-demand ERC. The HelPSCreen score demonstrated high diagnostic accuracy for identifying PSC patients. Conclusion: This study emphasises the critical need for targeted surveillance and early diagnostic strategies in PSC patients, given their significantly elevated risk of cholangiocarcinoma. Scheduled ERC with brush cytology proved to be the most effective surveillance strategy for improving clinical outcomes. The HelPSCreen score offers a simple scoring system to screen the probability of PSC. Further research and validation of these findings will be essential for optimising the management of PSC, particularly early detection of CCA

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