Long‐term outcomes of primary sclerosing cholangitis: an Australian non‐transplant tertiary hospital perspective

2019 ◽  
Vol 49 (3) ◽  
pp. 323-327 ◽  
Author(s):  
Elliot Freeman ◽  
Ammar Majeed ◽  
William Kemp ◽  
Stuart K. Roberts
Keyword(s):  
Primary Sclerosing Cholangitis ◽  
Sclerosing Cholangitis ◽  
Tertiary Hospital ◽  
Long Term Outcomes ◽  
Hospital Perspective

Long‐term outcomes of pediatric‐onset primary sclerosing cholangitis: A single‐center experience in Japan

2019 ◽  
Vol 49 (12) ◽  
pp. 1386-1397
Author(s):  
Shuichiro Umetsu ◽  
Kenji Notohara ◽  
Takahiro Nakazawa ◽  
Tomoyuki Tsunoda ◽  
Tsuyoshi Sogo ◽  
...  
Keyword(s):  
Primary Sclerosing Cholangitis ◽  
Sclerosing Cholangitis ◽  
Single Center ◽  
Long Term Outcomes ◽  
Single Center Experience ◽  
Pediatric Onset

Long-term outcomes of positive fluorescence in situ hybridization tests in primary sclerosing cholangitis

Hepatology ◽  
2009 ◽  
Vol 51 (1) ◽  
pp. 174-180 ◽  
Author(s):  
Sanjay Y. Bangarulingam ◽  
Einar Bjornsson ◽  
Felicity Enders ◽  
Emily G. Barr Fritcher ◽  
Gregory Gores ◽  
...  
Keyword(s):  
In Situ Hybridization ◽  
Fluorescence In Situ Hybridization ◽  
Primary Sclerosing Cholangitis ◽  
Sclerosing Cholangitis ◽  
Long Term Outcomes

scholarly journals S62 Predictors of Long-Term Outcomes of Primary Sclerosing Cholangitis Based on Cholangioscopic Features

2021 ◽  
Vol 116 (1) ◽  
pp. S27-S27
Author(s):  
Bernardo Guevara ◽  
Stephanie L. Yan ◽  
Jeffrey Fine ◽  
Sooraj Tejaswi ◽  
FACG ◽  
...  
Keyword(s):  
Primary Sclerosing Cholangitis ◽  
Sclerosing Cholangitis ◽  
Long Term Outcomes

scholarly journals A206 EVOLUTION OF PEDIATRIC AUTOIMMUNE CHOLANGITIS AND PRIMARY SCLEROSING CHOLANGITIS INTO ADULTHOOD

2021 ◽  
Vol 4 (Supplement_1) ◽  
pp. 234-236
Author(s):  
P Willems ◽  
J Hercun ◽  
C Vincent ◽  
F Alvarez
Keyword(s):  
Primary Sclerosing Cholangitis ◽  
Sclerosing Cholangitis ◽  
Response To Treatment ◽  
Similar Proportion ◽  
Autoimmune Cholangitis ◽  
Significant Difference ◽  
One Year ◽  
Liver Tests

Abstract Background The natural history of primary sclerosing cholangitis (PSC) in children seems to differ from PSC in adults. However, studies on this matter have been limited by short follow-up periods and inconsistent classification of patients with autoimmune cholangitis (AIC) (or overlap syndrome). Consequently, it remains unclear if long-term outcomes are affected by the clinical phenotype. Aims The aims of this is study are to describe the long-term evolution of PSC and AIC in a pediatric cohort with extension of follow-up into adulthood and to evaluate the influence of phenotype on clinical outcomes. Methods This is a retrospective study of patients with AIC or PSC followed at CHU-Sainte-Justine, a pediatric referral center in Montreal. All charts between January 1998 and December 2019 were reviewed. Patients were classified as either AIC (duct disease on cholangiography with histological features of autoimmune hepatitis) or PSC (large or small duct disease on cholangiography and/or histology). Extension of follow-up after the age of 18 was done for patients followed at the Centre hospitalier de l’Université de Montréal. Clinical features at diagnosis, response to treatment at one year and liver-related outcomes were compared. Results 40 patients (27 PSC and 13 AIC) were followed for a median time of 71 months (range 2 to 347), with 52.5% followed into adulthood. 70% (28/40) had associated inflammatory bowel disease (IBD) (78% PSC vs 54% AIC; p=0.15). A similar proportion of patients had biopsy-proven significant fibrosis at diagnosis (45% PSC vs 67% AIC; p=0.23). Baseline liver tests were similar in both groups. At diagnosis, all patients were treated with ursodeoxycholic acid. Significantly more patients with AIC (77% AIC vs 30 % PSC; p=0.005) were initially treated with immunosuppressive drugs, without a significant difference in the use of Anti-TNF agents (0% AIC vs 15% PSC; p= 0.12). At one year, 55% (15/27) of patients in the PSC group had normal liver tests versus only 15% (2/13) in the AIC group (p=0.02). During follow-up, more liver-related events (cholangitis, liver transplant and cirrhosis) were reported in the AIC group (HR=3.7 (95% CI: 1.4–10), p=0.01). Abnormal liver tests at one year were a strong predictor of liver-related events during follow-up (HR=8.9(95% CI: 1.2–67.4), p=0.03), while having IBD was not (HR=0.48 (95% CI: 0.15–1.5), p=0.22). 5 patients required liver transplantation with no difference between both groups (8% CAI vs 15% CSP; p=0.53). Conclusions Pediatric patients with AIC and PSC show, at onset, similar stage of liver disease with comparable clinical and biochemical characteristics. However, patients with AIC receive more often immunosuppressive therapy and treatment response is less frequent. AIC is associated with more liver-related events and abnormal liver tests at one year are predictor of bad outcomes. Funding Agencies None

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