Surveillance for #Hepatobiliary Cancers in Patients with Primary Sclerosing #Cholangitis
http://onlinelibrary.wiley.com/doi/10.1002/hep.29730/full
From 1995-2015, a total of 79 of 830 PSC patients were diagnosed with HBCa. Cumulative follow-up was 712 and 283 person-years pre- and post-HBCa diagnosis, respectively. Seventy-eight percent (54/79) developed CCA, 21% (17/79) HCC, 6% (5/79) GBCa, 3% (2/79) both CCA and HCC, and 1% (1/79) both HCC and GBCa. Fifty-one percent (40/79) were under surveillance and 49% (39/79) were not. Patients in the surveillance group had significantly higher 5-year overall survival (68% vs. 20%, respectively; p<0.001) and significantly lower 5-year probability of experiencing an HBCa-related adverse event (32% versus 75%, respectively; p<0.001), compared with the no-surveillance group.
Conclusion: This study demonstrates that surveillance for HBCa significantly improves outcomes, including survival, in patients with PSC
http://onlinelibrary.wiley.com/doi/10.1002/hep.29730/full
From 1995-2015, a total of 79 of 830 PSC patients were diagnosed with HBCa. Cumulative follow-up was 712 and 283 person-years pre- and post-HBCa diagnosis, respectively. Seventy-eight percent (54/79) developed CCA, 21% (17/79) HCC, 6% (5/79) GBCa, 3% (2/79) both CCA and HCC, and 1% (1/79) both HCC and GBCa. Fifty-one percent (40/79) were under surveillance and 49% (39/79) were not. Patients in the surveillance group had significantly higher 5-year overall survival (68% vs. 20%, respectively; p<0.001) and significantly lower 5-year probability of experiencing an HBCa-related adverse event (32% versus 75%, respectively; p<0.001), compared with the no-surveillance group.
Conclusion: This study demonstrates that surveillance for HBCa significantly improves outcomes, including survival, in patients with PSC
A PROMISING MEDIUM-TERM FOLLOW-UP OF PEDIATRIC #SCLEROSING #CHOLANGITIS: MILD PHENOTYPE OR EARLY DIAGNOSIS?
http://onlinelibrary.wiley.com/doi/10.1111/hepr.13059/abstract
Among 45 patients (median age: 10.4 years, 73.4% males) 29 (64.4%) were asymptomatic at presentation. Twenty patients (44%) had a concomitant inflammatory bowel disease (SC/IBD). Autoimmune features (ASC) were found in 20 patients (44%). Liver biopsy showed severe fibrosis or cirrhosis in 32% of cases. Patients with SC alone have a higher rate of interface hepatitis at liver biopsy than SC/IBD ones. All children received ursodeoxycholic acid (UDCA) at diagnosis, and in 17 steroids and/or azathioprine were associated. After a mean follow-up of 8.7 ± 5.6 years, all patients were alive and 7 developed at least one liver-related complication. At the end of follow-up, 10 patients stopped immunosuppressants and 2 had no therapy. Only 2 patients underwent liver transplantation. Complication-free survival did not differ between SC/IBD and SC patients while it was longer in patients without autoimmune features.
Conclusions
In our early-diagnosed population, the 9-year survival with native liver was better than what reported in other studies. About 15% of patients developed liver-related disease complications, less than previous reports. Long-term course of SC was negatively influenced by the presence of autoimmune features but not by concomitant IBD.
http://onlinelibrary.wiley.com/doi/10.1111/hepr.13059/abstract
Among 45 patients (median age: 10.4 years, 73.4% males) 29 (64.4%) were asymptomatic at presentation. Twenty patients (44%) had a concomitant inflammatory bowel disease (SC/IBD). Autoimmune features (ASC) were found in 20 patients (44%). Liver biopsy showed severe fibrosis or cirrhosis in 32% of cases. Patients with SC alone have a higher rate of interface hepatitis at liver biopsy than SC/IBD ones. All children received ursodeoxycholic acid (UDCA) at diagnosis, and in 17 steroids and/or azathioprine were associated. After a mean follow-up of 8.7 ± 5.6 years, all patients were alive and 7 developed at least one liver-related complication. At the end of follow-up, 10 patients stopped immunosuppressants and 2 had no therapy. Only 2 patients underwent liver transplantation. Complication-free survival did not differ between SC/IBD and SC patients while it was longer in patients without autoimmune features.
Conclusions
In our early-diagnosed population, the 9-year survival with native liver was better than what reported in other studies. About 15% of patients developed liver-related disease complications, less than previous reports. Long-term course of SC was negatively influenced by the presence of autoimmune features but not by concomitant IBD.
Wiley
“A PROMISING MEDIUM‐TERM FOLLOW‐UP OF PEDIATRIC SCLEROSING CHOLANGITIS: MILD PHENOTYPE OR EARLY DIAGNOSIS?”
Sclerosing cholangitis (SC) is a chronic cholestatic liver disease whose diagnosis is being increasingly recognized in childhood. Long‐term course and prognosis of pediatric SC is poorly described.We reviewed...
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Factors Associated With #Recurrence of Primary Biliary #Cholangitis After Liver Transplantation and Effects on Graft and Patient Survival
https://www.gastrojournal.org/article/S0016-5085(18)35089-3/fulltext
PBC recurred in 22% of patients after 5 years and 36% after 10 years. Age at diagnosis <50 years (hazard ratio HR, 1.79; 95% CI, 1.36–2.36; P < .001), age at liver transplantation <60 years (HR, 1.39; 95% CI, 1.02–1.90; P = .04), use of tacrolimus (HR, 2.31; 95% CI, 1.72–3.10; P < .001), and biochemical markers of severe cholestasis (bilirubin ≥100 μmol or alkaline phosphatase >3-fold the upper limit of normal) at 6 months after liver transplantation (HR, 1.79; 95% CI, 1.16–2.76; P = .008) were associated with higher risk of PBC recurrence, whereas use of cyclosporine reduced risk of PBC recurrence (HR, 0.62; 95% CI, 0.46–0.82; P = .001). In multivariable Cox regression with time-dependent covariate, recurrence of PBC significantly associated with graft loss (HR, 2.01; 95% CI, 1.16–3.51; P = .01) and death (HR, 1.72; 95% CI, 1.11–2.65; P = .02).
Conclusions
Younger age at the time of diagnosis with PBC or at liver transplantation, tacrolimus use, and biochemical markers of cholestasis after liver transplantation are associated with PBC recurrence. PBC recurrence reduces odds of graft and patient survival. Strategies are needed to prevent PBC recurrence or reduce its negative effects.
Factors Associated With #Recurrence of Primary Biliary #Cholangitis After Liver Transplantation and Effects on Graft and Patient Survival
https://www.gastrojournal.org/article/S0016-5085(18)35089-3/fulltext
PBC recurred in 22% of patients after 5 years and 36% after 10 years. Age at diagnosis <50 years (hazard ratio HR, 1.79; 95% CI, 1.36–2.36; P < .001), age at liver transplantation <60 years (HR, 1.39; 95% CI, 1.02–1.90; P = .04), use of tacrolimus (HR, 2.31; 95% CI, 1.72–3.10; P < .001), and biochemical markers of severe cholestasis (bilirubin ≥100 μmol or alkaline phosphatase >3-fold the upper limit of normal) at 6 months after liver transplantation (HR, 1.79; 95% CI, 1.16–2.76; P = .008) were associated with higher risk of PBC recurrence, whereas use of cyclosporine reduced risk of PBC recurrence (HR, 0.62; 95% CI, 0.46–0.82; P = .001). In multivariable Cox regression with time-dependent covariate, recurrence of PBC significantly associated with graft loss (HR, 2.01; 95% CI, 1.16–3.51; P = .01) and death (HR, 1.72; 95% CI, 1.11–2.65; P = .02).
Conclusions
Younger age at the time of diagnosis with PBC or at liver transplantation, tacrolimus use, and biochemical markers of cholestasis after liver transplantation are associated with PBC recurrence. PBC recurrence reduces odds of graft and patient survival. Strategies are needed to prevent PBC recurrence or reduce its negative effects.
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Factors Associated With Outcomes of Patients With Primary Sclerosing #Cholangitis and Development and Validation of a #Risk Scoring System
https://aasldpubs.onlinelibrary.wiley.com/doi/abs/10.1002/hep.30479
Serum alkaline phosphatase ≥2.4×ULN at 1 year post diagnosis, was predictive of 10‐year outcome (HR=3.05, C=0.63, median transplant‐free survival 63 versus 108 months, p<0.0001), as was the presence of extra‐hepatic biliary disease (HR=1.45, p=0.01). We developed two risk scoring systems based upon age, values of bilirubin, alkaline phosphatase, albumin, platelets, presence of extra‐hepatic biliary disease and variceal haemorrhage, which predicted 2‐ and 10‐year outcome with good discrimination (C=0.81 and 0.80 respectively). Both UK‐PSC risk scores were well‐validated in our external cohort, and out‐performed the Mayo and APRI scores (C=0.75 and 0.63 respectively). Whilst heterozygosity for the previously validated HLA‐DR*03:01 risk allele predicted increased risk of adverse outcome (HR=1.33, p=.001), its addition did not improve the predictive accuracy the UK‐PSC risk scores.
Conclusions
Our analyses, based upon a detailed clinical evaluation of a large representative cohort of participants with PSC, furthers our understanding of clinical risk markers and reports the development and validation of a real‐world scoring system to identify those patients most likely to die or require liver transplantation.
Factors Associated With Outcomes of Patients With Primary Sclerosing #Cholangitis and Development and Validation of a #Risk Scoring System
https://aasldpubs.onlinelibrary.wiley.com/doi/abs/10.1002/hep.30479
Serum alkaline phosphatase ≥2.4×ULN at 1 year post diagnosis, was predictive of 10‐year outcome (HR=3.05, C=0.63, median transplant‐free survival 63 versus 108 months, p<0.0001), as was the presence of extra‐hepatic biliary disease (HR=1.45, p=0.01). We developed two risk scoring systems based upon age, values of bilirubin, alkaline phosphatase, albumin, platelets, presence of extra‐hepatic biliary disease and variceal haemorrhage, which predicted 2‐ and 10‐year outcome with good discrimination (C=0.81 and 0.80 respectively). Both UK‐PSC risk scores were well‐validated in our external cohort, and out‐performed the Mayo and APRI scores (C=0.75 and 0.63 respectively). Whilst heterozygosity for the previously validated HLA‐DR*03:01 risk allele predicted increased risk of adverse outcome (HR=1.33, p=.001), its addition did not improve the predictive accuracy the UK‐PSC risk scores.
Conclusions
Our analyses, based upon a detailed clinical evaluation of a large representative cohort of participants with PSC, furthers our understanding of clinical risk markers and reports the development and validation of a real‐world scoring system to identify those patients most likely to die or require liver transplantation.