Przewlekła stwardniająca cholangitis
Rokowania, prognozy i postęp choroby

Przewlekłe stwardniające cholangitis (PSC) to postępująca choroba wątroby charakteryzująca się zapaleniem i destrukcją dróg żółciowych, z medianą przeżycia około 9,6 lat od diagnozy i 10-letnim przeżyciem na poziomie około 65%. Rokowanie zależy od wielu czynników, w tym wieku, płci, typu PSC (np. małych przewodów), poziomu fosfatazy alkalicznej (ALP) poniżej 1,5x górnej granicy normy po 1-2 latach oraz obecności dominujących zwężeń i marskości wątroby. Modele prognostyczne, takie jak Mayo Risk Score, AOM oraz PREsTo (z wartością statystyki C ≥ 0,90), umożliwiają ocenę ryzyka zgonu lub konieczności przeszczepu wątroby, uwzględniając parametry biochemiczne (bilirubina, albumina, ALP, AST), wiek, liczbę płytek, hemoglobinę, sód oraz czas od rozpoznania. Dodatkowo, aktywność autotaksyny (ATX) w surowicy koreluje z ciężkością choroby i przeżyciem bez przeszczepu, a wskaźniki ANALI i PFS oparte na MRI oferują nieinwazyjne narzędzia prognostyczne z wysoką wartością predykcyjną ujemną (~80-90%).

  1. Prognostyczne wskaźniki w przewlekłej stwardniającej cholangitis
    1. Czynniki prognostyczne
    2. Biomarkery i modele prognostyczne
    3. Ryzyko rozwoju cholangiocarcinoma
    4. Przeszczepianie wątroby
    5. Wnioski
    6. Kolejne rozdziały

Prognostyczne wskaźniki w przewlekłej stwardniającej cholangitis

Przewlekłe stwardniające cholangitis (Primary Sclerosing Cholangitis, PSC) jest postępującą chorobą wątroby charakteryzującą się zapaleniem i destrukcją wewnątrz- i/lub zewnątrzwątrobowych dróg żółciowych. Choroba ma zróżnicowany przebieg kliniczny, a jej przewidywalność stanowi istotne wyzwanie kliniczne. Szacowana mediana przeżycia od momentu diagnozy do zgonu lub przeszczepu wątroby wynosi około 9,6 lat, a szacowane 10-letnie przeżycie dla pacjentów z PSC to około 65%.12 Po rozpoznaniu średnia przewidywana długość życia waha się między 10 a 20 lat.3

Czynniki prognostyczne

Przebieg kliniczny PSC jest zróżnicowany między poszczególnymi pacjentami i podtypami choroby. Zidentyfikowano szereg czynników związanych z lepszym lub gorszym rokowaniem:4

Korzystne czynniki prognostyczne obejmują:

  • Bezobjawowy początek choroby (choć z czasem u większości pacjentów rozwijają się objawy)
  • Młodszy wiek w momencie diagnozy
  • Płeć żeńska
  • PSC małych przewodów żółciowych (lepsze rokowanie niż klasyczna postać choroby, mniejsze ryzyko rozwoju cholangiocarcinoma)
  • Poziom fosfatazy alkalicznej (ALP) niższy niż 1,5 razy górna granica normy po 1-2 latach od diagnozy

    • 5

Niekorzystne czynniki prognostyczne obejmują:

  • Rozległe zwężenia wewnątrz- lub zewnątrzwątrobowych przewodów żółciowych
  • Dominujące zwężenia (dominant stenosis)
  • Nawracające zapalenie dróg żółciowych
  • Współistniejące wrzodziejące zapalenie jelita grubego (w porównaniu z chorobą Leśniowskiego-Crohna lub brakiem IBD)
  • Zaburzenia funkcji syntetycznej wątroby
  • Marskość wątroby z nadciśnieniem wrotnym
  • Utrzymujące się podwyższenie stężenia bilirubiny dłużej niż 3 miesiące od rozpoznania
  • Hepatomegalia
  • Splenomegalia

    • 67

Pogorszenie cholestazy wiąże się z gorszymi wynikami, w tym zwiększonym ryzykiem przeszczepu wątroby, nowotworów wątrobowo-żółciowych i zgonu.8

Biomarkery i modele prognostyczne

Z uwagi na konieczność przewidywania przebiegu PSC, opracowano szereg modeli prognostycznych:9

Mayo Risk Score (MRS)

Jest to najczęściej stosowany system oceny, uwzględniający pomiary stężenia bilirubiny, AST i albuminy w surowicy, wiek pacjenta oraz obecność krwawienia z żylaków przełyku. Mayo Risk Score był wykorzystywany głównie do szacowania krótkoterminowego przeżycia w schyłkowej niewydolności wątroby u pacjentów z PSC, jednak nie był w stanie przewidzieć konieczności przeszczepu wątroby.1011

Amsterdam-Oxford Model (AOM)

Główną różnicą tego nowszego modelu jest oparcie na populacyjnej kohorcie oraz możliwość przewidywania zarówno krótko- jak i długoterminowych konsekwencji, takich jak zgon i/lub przeszczep wątroby. AOM wykazuje wystarczającą wartość dyskryminacyjną oraz odpowiednią i dobrą dokładność prognostyczną dla rokowania PSC zarówno w momencie diagnozy, jak i podczas okresu obserwacji, co czyni go przydatnym narzędziem w codziennej praktyce klinicznej.12

PREsTo Model

Jest to jeden z najnowszych modeli prognostycznych pozwalających dokładnie przewidzieć dekompensację wątroby u pacjentów z PSC w ciągu 5 lat. Model PREsTo został opracowany na podstawie danych około 500 pacjentów z ośrodka referencyjnego i zwalidowany na około 300 pacjentach w kohorcie międzynarodowej przy użyciu techniki opartej na uczeniu maszynowym. Składa się z dziewięciu zmiennych: bilirubiny, albuminy, fosfatazy alkalicznej w surowicy (ALP), płytek krwi, aminotransferazy asparaginianowej (AST), hemoglobiny, sodu, wieku pacjenta oraz liczby lat od rozpoznania PSC. Model ten znacznie przewyższa obecne systemy oceny prognostycznej z wartością statystyki C wynoszącą co najmniej 0,90.13

Autotaxin jako biomarker

Wykazano, że aktywność autotaksyny (ATX) w surowicy wiąże się z ciężkością choroby wątroby i przeżyciem bez przeszczepu. Pacjenci osiągający punkt końcowy (przeszczep wątroby lub zgon) wykazywali wyższą aktywność autotaksyny w porównaniu z pozostałymi pacjentami. Analizy przeżycia Kaplana-Meiera wykazały silny związek między zwiększoną aktywnością autotaksyny a krótszym przeżyciem bez przeszczepu wątroby. Zwiększona aktywność autotaksyny w surowicy wiąże się ze zmniejszonym przeżyciem bez przeszczepu wątroby niezależnie od wskaźnika ryzyka Mayo i markerów zapalenia i włóknienia.1415

Modele obrazowe (ANALI, PFS)

Wskaźniki ANALI (z kontrastem gadolinowym lub bez) oraz wskaźnik potencjalnego zwężenia czynnościowego (potential functional stricture, PFS) oparte na badaniach MRI mogą nieinwazyjnie przewidywać rokowanie u pacjentów z PSC. Analiza wieloczynnikowa zidentyfikowała PFS, wszystkie trzy wskaźniki ANALI i zrewidowany Mayo Risk Score jako niezależne czynniki ryzyka dla punktów końcowych (HR odpowiednio 3,12, p<0,001; 6,12, p<0,001; 3,56, p<0,001; 3,59, p<0,001 i 4,13, p<0,001).16

Wartości predykcyjne ujemne (NPV) dla wszystkich wskaźników ANALI były bardzo wysokie (około 90%), a dla PFS około 80%, podczas gdy wartości predykcyjne dodatnie (PPV) nie przekraczały 50-60%. Tak wysokie NPV mogą pomóc klinicystom zidentyfikować pacjentów z PSC, u których prawdopodobieństwo wystąpienia niekorzystnego zdarzenia jest niskie, co może pomóc uniknąć niepotrzebnych inwazyjnych testów i leczenia.17

Amsterdam Cholangiographic Scoring System

System klasyfikacji cholangiograficznej zmian wewnątrz- i zewnątrzwątrobowych dróg żółciowych przy użyciu ERCP, który pozwala szacować średnio- i długoterminowe rokowanie w PSC, został zwalidowany jako model prognostyczny w PSC.18

Ryzyko rozwoju cholangiocarcinoma

Cholangiocarcinoma (CCA) jest najczęstszym nowotworem złośliwym u pacjentów z PSC, występującym w 2-8% przypadków i stanowiącym główną przyczynę zgonów u tych pacjentów.19 Oszacowane roczne ryzyko rozwoju CCA wynosi między 0,5% a 1,5%. Pacjenci z PSC mają 5- i 10-letnie skumulowane ryzyko rozwoju CCA wynoszące odpowiednio około 7% i 6-11%.20

Częstość występowania CCA u pacjentów z PSC jest wysoce zmienna – wcześniejsze badania podawały szeroki zakres od 4% do 36%. Nowsze badania (na większych i wieloośrodkowych populacjach) odnotowały niższą częstość występowania CCA w PSC, rejestrując zakres od 2% do 8%.21

Pomimo znaczącego dwukrotnego zmniejszenia śmiertelności u pacjentów z PSC, którzy poddawani są regularnym badaniom obrazowym, CCA nadal odpowiada za prawie 30% wszystkich zgonów związanych z PSC, będąc główną przyczyną zgonów u tych pacjentów. Wśród osób z PSC-CCA, do 80% pacjentów umiera w ciągu pierwszego roku po rozpoznaniu.22

Pozytywny wpływ nadzoru radiologicznego dla pacjentów z PSC został już udowodniony przez różne badania, wykazujące lepsze wyniki ze zmniejszeniem liczby zgonów związanych z nowotworami wątrobowo-żółciowymi u pacjentów poddanych przynajmniej rutynowej technice obrazowania.23

Ostatnio opracowano modele predykcyjne oparte na sztucznej inteligencji, danych klinicznych i laboratoryjnych dla przewidywania rozwoju CCA u pacjentów z PSC. Dłuższy czas trwania nieswoistych chorób zapalnych jelit (IBD), dłuższy czas trwania PSC i wyższe stężenie bilirubiny całkowitej okazały się statystycznie istotnie predykcyjne dla CCA. Modele oparte na AI wykazały znacznie lepszą skuteczność niż powszechnie stosowane wskaźniki ryzyka PSC.24

Przeszczepianie wątroby

Przeszczep wątroby jest jedynym znanym leczeniem zaawansowanego PSC prowadzącego do niewydolności wątroby, jednak choroba może nawrócić w przeszczepionej wątrobie.25

Analiza Europejskiego Rejestru Przeszczepów Wątroby (ELTR) obejmująca 1549 przeszczepów wątroby z powodu PSC zidentyfikowała nawrót PSC (rPSC) u 259 pacjentów (16,7%) po medianie obserwacji wynoszącej 60 miesięcy, co miało negatywny wpływ na przeżycie pacjentów.26 W innych badaniach odnotowano nawrót PSC w przeszczepionej wątrobie u około 20-30% pacjentów.27

Dane wskazują, że skumulowane przeżycie pacjentów z PSC, którzy przeszli przeszczep wątroby, nie zmniejszyło się znacząco po wdrożeniu alokacji opartej na modelu MELD.28 Współcześni pacjenci z PSC wykazują doskonałe 10-letnie wskaźniki przeżycia po przeszczepie, szacowane na 74,6%.29

Wskaźniki przeżycia przeszczepu wynosiły 55,4% po 5 latach i 32,8% po 10 latach po przeszczepie wątroby z powodu PSC. Nawrót PSC zdiagnozowano u 16 (40%) z 40 pacjentów, średnio 30 miesięcy (zakres 9-70 miesięcy) po przeszczepie wątroby. Skumulowana częstość występowania nawrotu PSC wynosiła 24,5% po 3 latach, 39,3% po 5 latach i 45,8% po 6 latach.30

Wśród 16 pacjentów, u których zdiagnozowano nawrót PSC, wskaźnik przeżycia przeszczepu wynosił 56,3% po 5 latach i 21,9% po 10 latach po nawrocie. Nawrót PSC wiązał się z wyższą śmiertelnością (83%) w porównaniu z grupą bez nawrotu (21%).3132

Utrzymywanie nieaktywnego statusu nieswoistej choroby zapalnej jelit może oferować ochronę przed nawrotem PSC po przeszczepie. Badanie zidentyfikowało kolektomię przed lub podczas przeszczepu wątroby jako czynnik ochronny przeciwko nawrotowi PSC.3334

Wnioski

Przewlekłe stwardniające cholangitis (PSC) jest postępującą chorobą wątroby o zróżnicowanym przebiegu klinicznym. Dla oceny rokowania opracowano szereg modeli prognostycznych, które mogą pomóc w przewidywaniu przebiegu choroby i planowaniu leczenia.

Najważniejszymi czynnikami prognostycznymi są wiek, niski poziom albuminy, utrzymujące się podwyższenie bilirubiny dłużej niż 3 miesiące, hepatomegalia, splenomegalia, dominujące zwężenie dróg żółciowych oraz zmiany w wewnątrz- i zewnątrzwątrobowych przewodach żółciowych. U niektórych pacjentów rozwój cholangiocarcinoma znacząco pogarsza rokowanie.

Przeszczep wątroby pozostaje jedyną opcją leczenia zaawansowanej choroby, jednak nawrót PSC w przeszczepionej wątrobie występuje u znaczącego odsetka pacjentów, co wpływa na długoterminowe rokowanie. Regularne monitorowanie pacjentów z PSC, szczególnie pod kątem rozwoju nowotworów dróg żółciowych, może przyczynić się do poprawy wyników leczenia.

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  1. 16.04.2026
  2. www.leksykon.com.pl

Materiały źródłowe

  • #1
    https://journals.lww.com/kidney360/00000434-200701000-00019.fulltext
    Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease with varying severity and progression. This study describes the natural history of PSC patients and evaluates the prognostic significance of clinical, biochemical, and cholangiographic findings constructing a novel prognostic model. […] The estimated median survival from the time of diagnosis to death or time of liver transplantation was 9.6 yr. […] Age, low albumin, persistent bilirubin elevation longer than 3 months, hepatomegaly, splenomegaly, dominant bile duct stenosis, and intra- and extrahepatic ductal changes at the time of diagnosis were found to be independent risk factors correlating with poor prognosis and were used to construct a new prognostic model. […] A persistent bilirubin elevation for longer than 3 months from the time of diagnosis could be identified as a novel marker correlating with a poor outcome. A new prognostic model was developed to predict progression of PSC, which may be useful in timing of liver transplantation.
  • #2 Primary sclerosing cholangitis – Rabiee – Translational Gastroenterology and Hepatology
    https://tgh.amegroups.org/article/view/6491/html
    PSC is a progressive disease, with evolution to biliary cirrhosis and malignancy in the majority of patients. The estimated 10-year survival for patients with PSC is approximately 65%. […] Though patients who are asymptomatic have a better prognosis than those with symptoms at diagnosis, symptoms often develop over time. However, there is significant variation among individuals and between different subtypes. For example, patients with small duct PSC disease generally have better outcomes than those with classic disease and do not seem to develop CCA, unless the disease has progressed to large-duct PSC. Other favorable prognostic factors include younger age at diagnosis, and female sex. On the other hand, poor prognostic factors include extensive intrahepatic or extrahepatic biliary strictures, DSs, recurrent cholangitis, UC [compared to Crohns disease or no IBD], evidence of liver synthetic dysfunction and cirrhosis with portal hypertension.
  • #3 Primary Sclerosing Cholangitis: Symptoms, Treatment & Diagnosis
    https://my.clevelandclinic.org/health/diseases/23569-primary-sclerosing-cholangitis
    After diagnosis, the average life expectancy ranges between 10 and 20 years. […] A liver transplant can give you a new lease on life. However, in 15% to 20% of cases, PSC may return after a liver transplant. When this happens, the new liver may fail. The average life expectancy in this case is about nine months. […] Another factor that can affect life expectancy is cancer. If cancer develops as a complication of PSC, you may not be a good candidate for a liver transplant. In carefully selected cases, healthcare providers may attempt to treat cancer first with radiation or chemotherapy and then attempt a liver transplant.
  • #4 Primary sclerosing cholangitis – Rabiee – Translational Gastroenterology and Hepatology
    https://tgh.amegroups.org/article/view/6491/html
    PSC is a progressive disease, with evolution to biliary cirrhosis and malignancy in the majority of patients. The estimated 10-year survival for patients with PSC is approximately 65%. […] Though patients who are asymptomatic have a better prognosis than those with symptoms at diagnosis, symptoms often develop over time. However, there is significant variation among individuals and between different subtypes. For example, patients with small duct PSC disease generally have better outcomes than those with classic disease and do not seem to develop CCA, unless the disease has progressed to large-duct PSC. Other favorable prognostic factors include younger age at diagnosis, and female sex. On the other hand, poor prognostic factors include extensive intrahepatic or extrahepatic biliary strictures, DSs, recurrent cholangitis, UC [compared to Crohns disease or no IBD], evidence of liver synthetic dysfunction and cirrhosis with portal hypertension.
  • #5 Primary sclerosing cholangitis – Rabiee – Translational Gastroenterology and Hepatology
    https://tgh.amegroups.org/article/view/6491/html
    PSC is a progressive disease, with evolution to biliary cirrhosis and malignancy in the majority of patients. The estimated 10-year survival for patients with PSC is approximately 65%. […] Though patients who are asymptomatic have a better prognosis than those with symptoms at diagnosis, symptoms often develop over time. However, there is significant variation among individuals and between different subtypes. For example, patients with small duct PSC disease generally have better outcomes than those with classic disease and do not seem to develop CCA, unless the disease has progressed to large-duct PSC. Other favorable prognostic factors include younger age at diagnosis, and female sex. On the other hand, poor prognostic factors include extensive intrahepatic or extrahepatic biliary strictures, DSs, recurrent cholangitis, UC [compared to Crohns disease or no IBD], evidence of liver synthetic dysfunction and cirrhosis with portal hypertension.
  • #6 Primary sclerosing cholangitis – Rabiee – Translational Gastroenterology and Hepatology
    https://tgh.amegroups.org/article/view/6491/html
    PSC is a progressive disease, with evolution to biliary cirrhosis and malignancy in the majority of patients. The estimated 10-year survival for patients with PSC is approximately 65%. […] Though patients who are asymptomatic have a better prognosis than those with symptoms at diagnosis, symptoms often develop over time. However, there is significant variation among individuals and between different subtypes. For example, patients with small duct PSC disease generally have better outcomes than those with classic disease and do not seem to develop CCA, unless the disease has progressed to large-duct PSC. Other favorable prognostic factors include younger age at diagnosis, and female sex. On the other hand, poor prognostic factors include extensive intrahepatic or extrahepatic biliary strictures, DSs, recurrent cholangitis, UC [compared to Crohns disease or no IBD], evidence of liver synthetic dysfunction and cirrhosis with portal hypertension.
  • #7
    https://journals.lww.com/kidney360/00000434-200701000-00019.fulltext
    Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease with varying severity and progression. This study describes the natural history of PSC patients and evaluates the prognostic significance of clinical, biochemical, and cholangiographic findings constructing a novel prognostic model. […] The estimated median survival from the time of diagnosis to death or time of liver transplantation was 9.6 yr. […] Age, low albumin, persistent bilirubin elevation longer than 3 months, hepatomegaly, splenomegaly, dominant bile duct stenosis, and intra- and extrahepatic ductal changes at the time of diagnosis were found to be independent risk factors correlating with poor prognosis and were used to construct a new prognostic model. […] A persistent bilirubin elevation for longer than 3 months from the time of diagnosis could be identified as a novel marker correlating with a poor outcome. A new prognostic model was developed to predict progression of PSC, which may be useful in timing of liver transplantation.
  • #8 Primary sclerosing cholangitis – Rabiee – Translational Gastroenterology and Hepatology
    https://tgh.amegroups.org/article/view/6491/html
    Biomarkers to predict the pace of progression of any form of PSC have been described. One or 2 years after diagnosis, a serum ALP level of less than 1.5 times the ULN has been associated with better outcomes, regardless of treatment. Worsening cholestasis predicts poorer outcomes, including increased risk of LT, hepatobiliary cancer, and death. […] Multiple prognostic scoring systems that incorporate various clinical factors have been proposed. The most commonly used scoring system is the Mayo risk score (MRS), which is calculated based on measurements of serum bilirubin, AST, and albumin; the age of the patient and the presence of variceal bleeding. […] The Amsterdam Cholangiographic Scoring System, in which cholangiographic classification of intra- and extrahepatic biliary lesions using ERCP estimate medium- and long-term prognosis in PSC, has been validated as a prognostic model in PSC.
  • #9 A new prognostic model for primary sclerosing cholangitis
    https://pmc.ncbi.nlm.nih.gov/articles/PMC6883998/
    Primary sclerosing cholangitis (PSC) is a progressive liver disease characterized by destruction and inflammation of the intra-and/or extrahepatic biliary tract. […] For that reason, several risk models have been developed to predict the outcome. However, their utility remains controversial. […] The Mayo risk score has been used more frequently to estimate short term survival for the end stage liver disease of patients with PSC, unfortunately, it was not able to predict LT requirement. […] Recently the Amsterdam-Oxford model (AOM) was reported by of Vries. The main difference of the new model was being a population-based cohort and be able to predict both the short and the long-term consequences such as death and/or liver transplantation. […] In conclusion, we can say that AOM has sufficient discriminant value and adequate and good prediction accuracy for PSC prognosis at diagnosis and during the follow-up period. So, it can be useful for daily clinical practice.
  • #10 A new prognostic model for primary sclerosing cholangitis
    https://pmc.ncbi.nlm.nih.gov/articles/PMC6883998/
    Primary sclerosing cholangitis (PSC) is a progressive liver disease characterized by destruction and inflammation of the intra-and/or extrahepatic biliary tract. […] For that reason, several risk models have been developed to predict the outcome. However, their utility remains controversial. […] The Mayo risk score has been used more frequently to estimate short term survival for the end stage liver disease of patients with PSC, unfortunately, it was not able to predict LT requirement. […] Recently the Amsterdam-Oxford model (AOM) was reported by of Vries. The main difference of the new model was being a population-based cohort and be able to predict both the short and the long-term consequences such as death and/or liver transplantation. […] In conclusion, we can say that AOM has sufficient discriminant value and adequate and good prediction accuracy for PSC prognosis at diagnosis and during the follow-up period. So, it can be useful for daily clinical practice.
  • #11 Primary sclerosing cholangitis – Rabiee – Translational Gastroenterology and Hepatology
    https://tgh.amegroups.org/article/view/6491/html
    Biomarkers to predict the pace of progression of any form of PSC have been described. One or 2 years after diagnosis, a serum ALP level of less than 1.5 times the ULN has been associated with better outcomes, regardless of treatment. Worsening cholestasis predicts poorer outcomes, including increased risk of LT, hepatobiliary cancer, and death. […] Multiple prognostic scoring systems that incorporate various clinical factors have been proposed. The most commonly used scoring system is the Mayo risk score (MRS), which is calculated based on measurements of serum bilirubin, AST, and albumin; the age of the patient and the presence of variceal bleeding. […] The Amsterdam Cholangiographic Scoring System, in which cholangiographic classification of intra- and extrahepatic biliary lesions using ERCP estimate medium- and long-term prognosis in PSC, has been validated as a prognostic model in PSC.
  • #12 A new prognostic model for primary sclerosing cholangitis
    https://pmc.ncbi.nlm.nih.gov/articles/PMC6883998/
    Primary sclerosing cholangitis (PSC) is a progressive liver disease characterized by destruction and inflammation of the intra-and/or extrahepatic biliary tract. […] For that reason, several risk models have been developed to predict the outcome. However, their utility remains controversial. […] The Mayo risk score has been used more frequently to estimate short term survival for the end stage liver disease of patients with PSC, unfortunately, it was not able to predict LT requirement. […] Recently the Amsterdam-Oxford model (AOM) was reported by of Vries. The main difference of the new model was being a population-based cohort and be able to predict both the short and the long-term consequences such as death and/or liver transplantation. […] In conclusion, we can say that AOM has sufficient discriminant value and adequate and good prediction accuracy for PSC prognosis at diagnosis and during the follow-up period. So, it can be useful for daily clinical practice.
  • #13 Primary sclerosing cholangitis – Rabiee – Translational Gastroenterology and Hepatology
    https://tgh.amegroups.org/article/view/6491/html
    The PREsTo is one of the newest prognostic models to accurately predict liver decompensation patients with PSC at 5 years. The PREsTo was derived from approximately 500 patients from a referral center and validated in about 300 patients in an international cohort, using a machine-based learning technique, and consists of nine variables: bilirubin, albumin, serum ALP times the ULN, platelets, aspartate aminotransferase (AST), hemoglobin, sodium, patient age, and number of years since PSC was diagnosed. It significantly outperforms current prediction scoring systems with a C statistic of at least 0.90.
  • #14 Autotaxin activity predicts transplant-free survival in primary sclerosing cholangitis | Scientific Reports
    https://www.nature.com/articles/s41598-019-44762-7
    Autotaxin has been associated with liver disease severity and transplant-free survival. […] Patients reaching an end-point, liver transplantation or death, showed higher autotaxin activity compared with the other patients. […] Kaplan-Meier survival analyses showed a strong association between increasing autotaxin activity and shorter liver transplant-free survival. […] In conclusion, increased serum autotaxin activity is associated with reduced liver transplant-free survival independent from Mayo risk score and markers of inflammation and fibrosis. […] High autotaxin activity is associated with poor prognosis in PSC patients. […] Patients who reached an end-point during follow-up showed higher ATX activity compared with the other patients in both cohorts. […] When stratifying patients according to autotaxin tertiles, there was a strong association between increasing autotaxin activity and shorter liver transplant-free survival in both cohorts.
  • #15 Autotaxin activity predicts transplant-free survival in primary sclerosing cholangitis | Scientific Reports
    https://www.nature.com/articles/s41598-019-44762-7
    High autotaxin levels are associated with poor survival in subgroups with both low IL-8 values, and high IL-8 values. […] High autotaxin as defined by the validated cut-off was also associated with an increased risk of liver transplantation or death, independent from Mayo risk score, the in-house ELF score and IL-8. […] Increased serum autotaxin activity is associated with reduced liver transplant-free survival in PSC patients.
  • #16
    https://link.springer.com/article/10.1007/s00330-024-10787-4
    Our aim was twofold. First, to validate Anali scores with and without gadolinium (ANALIGd and ANALINoGd) in primary sclerosing cholangitis (PSC) patients. Second, to compare the ANALIs prognostic ability with the recently-proposed potential functional stricture (PFS). […] Univariate Cox regression showed that outcomes for decompensated cirrhosis, orthotopic liver transplantation or death significantly correlated with PFS (HR (hazard ratio)=3.15, p0.001), ANALINoGd (HR=6.42, p0.001), ANALIGdHBP (HR=3.66, p0.001) and ANALIGdAP (HR=3.79, p0.001). Multivariate analysis identified the PFS, all three ANALI scores, and Revised Mayo Risk Score as independent risk factors for outcomes (HR 3.12, p0.001; 6.12, p0.001; 3.56, p0.001;3.59, p0.001; and 4.13, p0.001, respectively). […] ANALINoGd and GA-MRI-derived ANALI scores and PFS could noninvasively predict outcomes in PSC patients.
  • #17
    https://link.springer.com/article/10.1007/s00330-024-10787-4
    The combined use of Anali scores and the potential functional stricture (PFS), both derived from unenhanced-, and gadoxetic acid enhanced-MRI, could be applied as a diagnostic and prognostic imaging surrogate for counselling and monitoring primary sclerosing cholangitis patients. […] We found that PFS, and all three ANALI scores (ANALINoGd, ANALIGdAP, ANALIGdHBP) could non-invasively predict PSC outcomes, i.e., liver-related death, the need for OLT, and decompensation. […] The predictive value of these binary single- and mean-reader ANALI scores (low- and high-risk) was statistically significant, confirming the value of ANALI scores as an outcome prognosticator. […] The mean NPVs were very high (i.e., ca 90%) for all ANALI scores and ca 80% for the PFS, while the PPVs were never more than 5060%. Such high NPVs can help clinicians identify PSC patients who are unlikely to have an event, i.e., at low risk. This can help to avoid unnecessary invasive tests and treatments.
  • #18 Primary sclerosing cholangitis – Rabiee – Translational Gastroenterology and Hepatology
    https://tgh.amegroups.org/article/view/6491/html
    Biomarkers to predict the pace of progression of any form of PSC have been described. One or 2 years after diagnosis, a serum ALP level of less than 1.5 times the ULN has been associated with better outcomes, regardless of treatment. Worsening cholestasis predicts poorer outcomes, including increased risk of LT, hepatobiliary cancer, and death. […] Multiple prognostic scoring systems that incorporate various clinical factors have been proposed. The most commonly used scoring system is the Mayo risk score (MRS), which is calculated based on measurements of serum bilirubin, AST, and albumin; the age of the patient and the presence of variceal bleeding. […] The Amsterdam Cholangiographic Scoring System, in which cholangiographic classification of intra- and extrahepatic biliary lesions using ERCP estimate medium- and long-term prognosis in PSC, has been validated as a prognostic model in PSC.
  • #19 Primary Sclerosing Cholangitis-Associated Cholangiocarcinoma: From Pathogenesis to Diagnostic and Surveillance Strategies
    https://www.mdpi.com/2072-6694/15/20/4947
    Cholangiocarcinoma (CCA) is the most common malignancy in patients with primary sclerosing cholangitis (PSC), accounting for 2–8% of cases and being the leading cause of death in these patients. […] The assessed annual risk ranges between 0.5% and 1.5%. According to that, PSC patients are affected by 5- and 10-year cumulative risk of developing CCA around 7% and 6–11%, respectively. […] Despite the significant two-fold reduction of mortality in PSC patients who undergo scheduled imaging surveillance, CCA still accounts for almost 30% of all PSC-related deaths, being the main cause of death in these patients. […] Furthermore, among those affected by PSC-CCA, up to 80% of patients die within the first year after diagnosis. […] The frequency of CCA in patients with PSC is highly variable, as past studies reported a wide range between 4% and 36%.
  • #20 Primary Sclerosing Cholangitis-Associated Cholangiocarcinoma: From Pathogenesis to Diagnostic and Surveillance Strategies
    https://www.mdpi.com/2072-6694/15/20/4947
    Cholangiocarcinoma (CCA) is the most common malignancy in patients with primary sclerosing cholangitis (PSC), accounting for 2–8% of cases and being the leading cause of death in these patients. […] The assessed annual risk ranges between 0.5% and 1.5%. According to that, PSC patients are affected by 5- and 10-year cumulative risk of developing CCA around 7% and 6–11%, respectively. […] Despite the significant two-fold reduction of mortality in PSC patients who undergo scheduled imaging surveillance, CCA still accounts for almost 30% of all PSC-related deaths, being the main cause of death in these patients. […] Furthermore, among those affected by PSC-CCA, up to 80% of patients die within the first year after diagnosis. […] The frequency of CCA in patients with PSC is highly variable, as past studies reported a wide range between 4% and 36%.
  • #21 Primary Sclerosing Cholangitis-Associated Cholangiocarcinoma: From Pathogenesis to Diagnostic and Surveillance Strategies
    https://www.mdpi.com/2072-6694/15/20/4947
    More recent studies (on larger and multicentric populations) reported a higher frequency of CCA in PSC, registering a range from 2% to 8%. […] The clinical presentation of PSC-CCA is difficult to recognize also because the clinical and radiological features are overlapping in the two diseases, and CCA could appear as a precipitating event in misdiagnosed PSC. […] However, the majority of CCA develops asymptomatically, representing an incidental event in up to 40% of all PSC-CCA, also discovered in liver explant pathology or at autopsy. […] The positive impact of radiological surveillance for PSC patients has already been proven by different studies, reporting better outcomes with a reduction in hepatobiliary cancers-related deaths in patients exposed to at least a routine imaging technique. […] Even without strong evidence, a closer surveillance with MRI/MRCP also including DWI, validated by an expert radiologist, could be a possible strategy for increasing early CCA diagnosis, allowing curative treatments, such as liver resection or LT.
  • #22 Primary Sclerosing Cholangitis-Associated Cholangiocarcinoma: From Pathogenesis to Diagnostic and Surveillance Strategies
    https://www.mdpi.com/2072-6694/15/20/4947
    Cholangiocarcinoma (CCA) is the most common malignancy in patients with primary sclerosing cholangitis (PSC), accounting for 2–8% of cases and being the leading cause of death in these patients. […] The assessed annual risk ranges between 0.5% and 1.5%. According to that, PSC patients are affected by 5- and 10-year cumulative risk of developing CCA around 7% and 6–11%, respectively. […] Despite the significant two-fold reduction of mortality in PSC patients who undergo scheduled imaging surveillance, CCA still accounts for almost 30% of all PSC-related deaths, being the main cause of death in these patients. […] Furthermore, among those affected by PSC-CCA, up to 80% of patients die within the first year after diagnosis. […] The frequency of CCA in patients with PSC is highly variable, as past studies reported a wide range between 4% and 36%.
  • #23 Primary Sclerosing Cholangitis-Associated Cholangiocarcinoma: From Pathogenesis to Diagnostic and Surveillance Strategies
    https://www.mdpi.com/2072-6694/15/20/4947
    More recent studies (on larger and multicentric populations) reported a higher frequency of CCA in PSC, registering a range from 2% to 8%. […] The clinical presentation of PSC-CCA is difficult to recognize also because the clinical and radiological features are overlapping in the two diseases, and CCA could appear as a precipitating event in misdiagnosed PSC. […] However, the majority of CCA develops asymptomatically, representing an incidental event in up to 40% of all PSC-CCA, also discovered in liver explant pathology or at autopsy. […] The positive impact of radiological surveillance for PSC patients has already been proven by different studies, reporting better outcomes with a reduction in hepatobiliary cancers-related deaths in patients exposed to at least a routine imaging technique. […] Even without strong evidence, a closer surveillance with MRI/MRCP also including DWI, validated by an expert radiologist, could be a possible strategy for increasing early CCA diagnosis, allowing curative treatments, such as liver resection or LT.
  • #24 Predicting cholangiocarcinoma in primary sclerosing cholangitis: using artificial intelligence, clinical and laboratory data | BMC Gastroenterology | Full Text
    https://bmcgastroenterol.biomedcentral.com/articles/10.1186/s12876-023-02759-7
    Primary sclerosing cholangitis (PSC) patients have a risk of developing cholangiocarcinoma (CCA). Establishing predictive models for CCA in PSC is important. The lifetime risk of CCA in PSC patients has been reported to be 7%13%. While somewhat treatable when detected early, CCA remains a significant cause of mortality in PSC patients, due to lack of clinically useful prediction tools. Ability to predict development of CCA in PSC patients could lead to better surveillance programs capable of identifying CCA at a curable stage resulting in improved outcomes. Longer IBD duration, longer PSC duration, and higher total bilirubin were statistically significantly predictive of CCA. In a large PSC cohort, we identified clinical and laboratory risk factors for CCA development and demonstrated the first AI based predictive models that performed significantly better than commonly used PSC risk scores. We showed that clinical variables and laboratory parameters predicted CCA significantly better than the commonly used risk scores. The results were generalizable over the course of PSC, showing similar performance at 2- and 5- years post PSC diagnosis.
  • #25 Primary sclerosing cholangitis – Symptoms and causes – Mayo Clinic
    https://www.mayoclinic.org/diseases-conditions/primary-sclerosing-cholangitis/symptoms-causes/syc-20355797
    In most people with primary sclerosing cholangitis, the disease progresses slowly. It can eventually lead to liver failure, repeated infections, and tumors of the bile duct or liver. A liver transplant is the only known cure for advanced primary sclerosing cholangitis, but the disease may recur in the transplanted liver in a small number of patients. […] Many people diagnosed with primary sclerosing cholangitis before they have symptoms continue to feel generally well for several years. But there’s no reliable way to predict how quickly or slowly the disease will progress for any individual. […] People with primary sclerosing cholangitis associated with inflammatory bowel disease have an increased risk of colon cancer. If you’ve been diagnosed with primary sclerosing cholangitis, your doctor may recommend testing for inflammatory bowel disease, even if you have no signs or symptoms, since the risk of colon cancer is elevated if you have both diseases.
  • #26 Transplantation for Primary Sclerosing Cholangitis: Outcomes and Recurrence
    https://www.mdpi.com/2077-0383/12/10/3405
    The analysis of the European Liver Transplant registry (ELTR) involving 1549 LT for PSC identified a recurrence of PSC (rPSC) in 259 patients (16.7%) after a median follow-up of 60 months, and this had a negative impact on patient survival. […] The re-transplant rate in our study was 13.1%, which is in line with the study of Berenguer, who reported a retransplant rate of 16.1%. […] This study identified colectomy before during LT to be protective against rPSC. […] The advantages of duct-to-duct reconstruction include a shorter operation time, lower infection rate, more physiologic enteric functions and easier endoscopic access to the biliary tract. […] However, the recurrence of PSC (rPSC) in the donor liver is observed in approximately 20–30% of patients.
  • #27 Transplantation for Primary Sclerosing Cholangitis: Outcomes and Recurrence
    https://www.mdpi.com/2077-0383/12/10/3405
    The analysis of the European Liver Transplant registry (ELTR) involving 1549 LT for PSC identified a recurrence of PSC (rPSC) in 259 patients (16.7%) after a median follow-up of 60 months, and this had a negative impact on patient survival. […] The re-transplant rate in our study was 13.1%, which is in line with the study of Berenguer, who reported a retransplant rate of 16.1%. […] This study identified colectomy before during LT to be protective against rPSC. […] The advantages of duct-to-duct reconstruction include a shorter operation time, lower infection rate, more physiologic enteric functions and easier endoscopic access to the biliary tract. […] However, the recurrence of PSC (rPSC) in the donor liver is observed in approximately 20–30% of patients.
  • #28
    https://link.springer.com/article/10.1007/s00423-014-1214-6
    Survival after liver transplantation (LTX) has decreased in Germany since the implementation of Model for end-stage liver disease (MELD)-based liver allocation. Primary sclerosing cholangitis (PSC) is known for its otherwise excellent outcome after LTX. […] Progress is stagnating in LTX for PSC. Current liver allocation for PSC patients should be reconsidered. […] The current study investigates the outcome of LTX for PSC in the pre-MELD and MELD era, taking subsequent changes of allocation policy into account and systematically compares PSC patients with other indications for LTX. […] Our data indicates that the cumulative survival of PSC patients who underwent LTX has not declined significantly after the implementation of MELD-based allocation. […] The data of this study underlines the conclusion that before and after the implementation of MELD-based allocation, there is no progress and rather stagnation in outcome after LTX for PSC, pointing to a lack of medical progress for these patients which is even more pronounced for patients with other indications for LTX who experience worse outcomes since the introduction of MELD-based allocation in Germany.
  • #29 Transplantation for Primary Sclerosing Cholangitis: Outcomes and Recurrence
    https://www.mdpi.com/2077-0383/12/10/3405
    Primary sclerosing cholangitis (PSC) is characterized by inflammation of the whole bile duct system. […] The ten-year survival rate was 74.6%. […] Patients with PSC show excellent 10-year survival rates. While the lab-MELD score significantly affected long term outcomes, donor characteristics did not affect survival rates. […] The leading causes of mortality are malignancies of the biliary tract or colon, accounting for about 40% of deaths in PSC patients, while end-stage liver failure accounts for 33% of deaths. […] Approximately 30% of patients have histologic progression to cirrhosis within 5 years. […] The occurrence of PSC recurrence was associated with a higher mortality (83%) compared to the group without recurrence (21%, p < 0.004). [...] The main causes of mortality were primary nonfunction, sepsis and hepatic artery thrombosis (each: n = 3; 27.3%).
  • #30
    https://journals.lww.com/transplantationdirect/fulltext/2017/12000/long_term_prognosis_and_recurrence_of_primary.7.aspx
    Primary sclerosing cholangitis (PSC) is a progressive cholestatic liver disease, with liver transplantation being the sole life-saving treatment for end-stage PSC-related liver disease. However, recurrence of PSC after liver transplantation is a common complication, with the risk factors for recurrence being controversial. […] The graft survival rates were 55.4% at 5 years and 32.8% at 10 years after liver transplantation for PSC. PSC recurrence was diagnosed in 16 (40%) of 40 patients, at a median 30 months (range, 9-70 months) after liver transplantation. The cumulative incidence rate of PSC recurrence was 24.5% at 3 years, 39.3% at 5 years, and 45.8% at 6 years. Among the 16 patients diagnosed with PSC recurrence, the graft survival rate was 56.3% at 5 years, and 21.9% at 10 years after the recurrence.
  • #31
    https://journals.lww.com/transplantationdirect/fulltext/2017/12000/long_term_prognosis_and_recurrence_of_primary.7.aspx
    Primary sclerosing cholangitis (PSC) is a progressive cholestatic liver disease, with liver transplantation being the sole life-saving treatment for end-stage PSC-related liver disease. However, recurrence of PSC after liver transplantation is a common complication, with the risk factors for recurrence being controversial. […] The graft survival rates were 55.4% at 5 years and 32.8% at 10 years after liver transplantation for PSC. PSC recurrence was diagnosed in 16 (40%) of 40 patients, at a median 30 months (range, 9-70 months) after liver transplantation. The cumulative incidence rate of PSC recurrence was 24.5% at 3 years, 39.3% at 5 years, and 45.8% at 6 years. Among the 16 patients diagnosed with PSC recurrence, the graft survival rate was 56.3% at 5 years, and 21.9% at 10 years after the recurrence.
  • #32 Transplantation for Primary Sclerosing Cholangitis: Outcomes and Recurrence
    https://www.mdpi.com/2077-0383/12/10/3405
    Primary sclerosing cholangitis (PSC) is characterized by inflammation of the whole bile duct system. […] The ten-year survival rate was 74.6%. […] Patients with PSC show excellent 10-year survival rates. While the lab-MELD score significantly affected long term outcomes, donor characteristics did not affect survival rates. […] The leading causes of mortality are malignancies of the biliary tract or colon, accounting for about 40% of deaths in PSC patients, while end-stage liver failure accounts for 33% of deaths. […] Approximately 30% of patients have histologic progression to cirrhosis within 5 years. […] The occurrence of PSC recurrence was associated with a higher mortality (83%) compared to the group without recurrence (21%, p < 0.004). [...] The main causes of mortality were primary nonfunction, sepsis and hepatic artery thrombosis (each: n = 3; 27.3%).
  • #33
    https://journals.lww.com/transplantationdirect/fulltext/2017/12000/long_term_prognosis_and_recurrence_of_primary.7.aspx
    PSC frequently recurred and progressed to graft failure after liver transplantation for PSC. Maintaining an inactive status of inflammatory bowel disease might offer protection against PSC recurrence. […] The rate of graft failure at 10 years after liver transplantation, estimated by Kaplan-Meier analysis, was 67.2%. The cumulative incidence rate of PSC recurrence at 10 years after liver transplantation, analyzed by a competing risk method in our cohort, was 45.8%. Sixteen (40%) of 40 patients experienced a recurrence of PSC at a median of 30 months after liver transplantation, with 15 (94%) of these patients progressing to graft failure, even in cases of retransplantation.
  • #34 Transplantation for Primary Sclerosing Cholangitis: Outcomes and Recurrence
    https://www.mdpi.com/2077-0383/12/10/3405
    The analysis of the European Liver Transplant registry (ELTR) involving 1549 LT for PSC identified a recurrence of PSC (rPSC) in 259 patients (16.7%) after a median follow-up of 60 months, and this had a negative impact on patient survival. […] The re-transplant rate in our study was 13.1%, which is in line with the study of Berenguer, who reported a retransplant rate of 16.1%. […] This study identified colectomy before during LT to be protective against rPSC. […] The advantages of duct-to-duct reconstruction include a shorter operation time, lower infection rate, more physiologic enteric functions and easier endoscopic access to the biliary tract. […] However, the recurrence of PSC (rPSC) in the donor liver is observed in approximately 20–30% of patients.

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