scholarly journals P812 Inflammatory bowel disease and hepatopathies: Beyond primary sclerosing cholangitis

2020 ◽  
Vol 14 (Supplement_1) ◽  
pp. S633-S634
Author(s):  
M B Sanchez ◽  
M J Etchevers ◽  
J A De Paula ◽  
R Gonzalez Sueyro ◽  
P Daffra ◽  
...  
Keyword(s):  
Liver Disease ◽  
Primary Sclerosing Cholangitis ◽  
Median Time ◽  
Sclerosing Cholangitis ◽  
Hospital Admissions ◽  
Primary Objective ◽  
Biliary Cirrhosis ◽  
Reference Centre ◽  
Inflammatory Bowel ◽  
Clinical Records

Abstract Background Inflammatory bowel diseases (IBD) and primary sclerosing cholangitis (PSC) are closely related. IBD-PSC has clinical characteristics that distinguish it from IBD without autoimmune liver disease (ALD). The association between autoimmune hepatitis (AH), overlapping syndrome (OS), primary biliary cirrhosis (PBC) and IBD is less known. Methods Clinical records of patients with IBD and PSC, AH, OS and PBC in a reference centre were reviewed retrospectively. Primary objective: to estimate the prevalence of ALD in patients with IBD. Secondary objectives: to establish the temporal gap between diagnosis of IBD and hepatopathy, severity of IBD, transplant requirement, displasia/colorectal carcinoma (CRC), and correlation between symptoms and endoscopic findings. All variables were compared according to liver disease. Results Of 1895 patients with IBD, 108 (5.7% [CI95% 4.7–6.8%]) had concomitant ALD, 78 PSC, 27 AH, 3 OS; no patient had PBC. 96.3% were associated with ulcerative colitis (UC). The prevalence of ALD in patients with UC was 7.6% (CI95% 6.3–9.2) and 1% (CI95% 0.3–2.5%) in patients with Crohn’s Disease. In patients with PSC, 56.4% were men, IBD diagnosis was previous than hepatopathy in 54.3% (median time 8.0 years), subsequent in 10% (7.9 years) and simultaneous, less than 1 year, in 35.7%. Conversely, in patients with AH, 55.6% were women, IBD was first diagnosed in 16% (median time 6.5 years), subsequent in 40% (5.3 years) and simultaneous in 44.0%. The median age at diagnosis was 32.5 (IQR 19.5–50.1) years for PSC and 13.1 (IQR 5.6–16.0) years for AH. Of 77 patients with available data, 97% had extensive colitis, without difference between PSC and AH. Patients with AH had more severe phenotype than PSC (Graphic 1). High grade dysplasia/CRC was detected in 5 patients (9.6%) all with PSC. Liver transplantation was required in 38.5% of PSC-UC patients and in 20% of AH-UC patients. Regarding endoscopic-clinical concordance, 52% of patients with moderate-severe endoscopic score were asymptomatic with similar behaviour in both hepatopathies. Conclusion PSC and AH were the most frequent ALD associated with IBD, mostly extensive UC. In contrast to PSC, AH tended to be diagnosed before intestinal disease and at an earlier age with higher biological and hospital admissions requirement. More than a half of our cohort had an important clinical-endoscopic dissociation, so we emphasise the relevance of the objective measurement of inflammation with colonoscopy in this particular group of patients

Primary Sclerosing Cholangitis

2015 ◽  
Author(s):  
Udayakumar Navaneethan
Keyword(s):  
Differential Diagnosis ◽  
Primary Sclerosing Cholangitis ◽  
Sclerosing Cholangitis ◽  
Biliary Tree ◽  
Biliary Cirrhosis ◽  
Extrahepatic Bile Ducts ◽  
Biliary Stasis ◽  
Inflammatory Bowel ◽  
Direct Peroral Cholangioscopy ◽  
Secondary Biliary Cirrhosis

Primary sclerosing cholangitis (PSC) is a chronic progressive cholestatic disease characterized by inflammation and fibrosis that may involve the entire biliary tree. The fibrosis causes diffuse narrowing of the intrahepatic and extrahepatic bile ducts, and the resulting biliary stasis leads to secondary biliary cirrhosis and associated complications. This review addresses PSC through its epidemiology, etiopathogenesis, diagnosis, differential diagnosis, management, complications, and prognosis. Figures show pouchoscopy, liver biopsy, and endoscopic retrograde cholangiopancreatography; direct peroral cholangioscopy visualization of cholangiocarcinoma; and algorithms depicting diagnosis of cholangiocarcinoma and screening for inflammatory bowel disease (IBD) in patients with PSC. Tables list characteristics of IBD in PSC, prevalence of antibodies in PSC, differential diagnosis of PSC, diagnostic approach in PSC, and risk of cancers in PSC. This review contains 6 highly rendered figures, 5 tables, and 91 references. 

scholarly journals Role of the Microbiota and Antibiotics in Primary Sclerosing Cholangitis

2013 ◽  
Vol 2013 ◽  
pp. 1-7 ◽  
Author(s):  
James H. Tabibian ◽  
Jayant A. Talwalkar ◽  
Keith D. Lindor
Keyword(s):  
Inflammatory Bowel Disease ◽  
Liver Disease ◽  
Primary Sclerosing Cholangitis ◽  
Sclerosing Cholangitis ◽  
Clinical Evidence ◽  
Cholestatic Liver Disease ◽  
Concise Review ◽  
Inflammatory Bowel ◽  
Considerable Morbidity

Primary sclerosing cholangitis (PSC) is an idiopathic, progressive, cholestatic liver disease with considerable morbidity and mortality and no established pharmacotherapy. In addition to the long-recognized association between PSC and inflammatory bowel disease, several lines of preclinical and clinical evidence implicate the microbiota in the etiopathogenesis of PSC. Here we provide a concise review of these data which, taken together, support further investigation of the role of the microbiota and antibiotics in PSC as potential avenues toward elucidating safe and effective pharmacotherapy for patients afflicted by this illness.

Autoimmunity in liver disease

2019 ◽  
pp. 245-252
Author(s):  
Gavin Spickett
Keyword(s):  
Liver Disease ◽  
Primary Biliary Cirrhosis ◽  
Autoimmune Hepatitis ◽  
Primary Sclerosing Cholangitis ◽  
Liver Diseases ◽  
Sclerosing Cholangitis ◽  
Biliary Cirrhosis

This chapter covers the presentation, immunogenetics, immunopathology, diagnosis, treatment, and testing for a range of liver diseases. Primary biliary cirrhosis, autoimmune hepatitis, and primary sclerosing cholangitis are described.

scholarly journals Novel Insights of Primary Sclerosing Cholangitis and Primary Biliary Cholangitis

2020 ◽  
Vol 25 (2) ◽  
pp. 107-117
Author(s):  
Dong-Won Ahn
Keyword(s):  
Liver Transplantation ◽  
Liver Disease ◽  
Primary Sclerosing Cholangitis ◽  
Liver Diseases ◽  
Sclerosing Cholangitis ◽  
Acute Cholangitis ◽  
Magnetic Resonance Cholangiopancreatography ◽  
Primary Biliary Cholangitis ◽  
Cholestatic Liver Disease ◽  
Biliary Cirrhosis

Primary sclerosing cholangitis (PSC) and primary biliary cholangitis (PBC) are immune-mediated chronic liver diseases. PSC is a rare disorder characterized by multi-focal bile duct strictures and progressive liver diseases, in which liver transplantation is required ultimately in most patients. Imaging studies such as magnetic resonance cholangiopancreatography have important role in diagnosis in most cases of PSC. PSC is usually accompanied by inflammatory bowel disease and there is a high risk of cholangiocarcinoma and colorectal cancer in PSC. No medical therapies have been proven to delay progression of PSC. Endoscopic intervention for tissue diagnosis or biliary drainage is frequently required in cases of PSC with dominant stricture, acute cholangitis, or clinically suspected cholangiocarcinoma. PBC is a chronic inflammatory autoimmune cholestatic liver disease, which when untreated will culminate in endstage biliary cirrhosis requiring liver transplantation. Diagnosis is usually based on the presence of serum liver tests indicative of a cholestatic hepatitis in association with circulating antimitochondrial antibodies. Patient presentation and course can be diverse in PBC and risk stratification is important to ensure all patients receive a personalised approach to their care. Medical therapy using ursodeoxycholic acid (UDCA) or obeticholic acid (OCA) has an important role to reduce the progression to end-stage liver disease in PBC.

scholarly journals Medical Management of Chronic Cholestatic Liver Diseases

2000 ◽  
Vol 14 (suppl d) ◽  
pp. 93D-98D ◽  
Author(s):  
Andrea A Gossard ◽  
Keith D Lindor
Keyword(s):  
Liver Disease ◽  
Primary Biliary Cirrhosis ◽  
Primary Sclerosing Cholangitis ◽  
Liver Diseases ◽  
Biliary Obstruction ◽  
Sclerosing Cholangitis ◽  
Cholestatic Liver Disease ◽  
Biliary Cirrhosis ◽  
Management Options ◽  
Extrahepatic Biliary Obstruction

The purpose of the present review is to discuss the diagnosis and management of cholestatic liver diseases. Differential diagnoses to consider are described, including causes of extrahepatic biliary obstruction such as gallstones, strictures, extrabiliary malignancies and pancreatitis. In addition, diseases that cause intrahepatic cholestasis such as primary biliary cirrhosis, primary sclerosing cholangitis, hepatocellular diseases and a variety of miscellaneous causes including drugs that may cause cholestasis are discussed. Primary biliary cirrhosis and primary sclerosing cholangitis are reviewed in detail, and management options are identified. The prognosis of patients with these diseases is discussed, and the Mayo Mathematical Models in Cholestatic Liver Disease for both primary biliary cirrhosis and primary sclerosing cholangitis are provided. Finally, management options for the complications of cholestasis are provided.

Timing, Management, and Outcomes of Liver Transplantation in Primary Sclerosing Cholangitis

2017 ◽  
Vol 37 (04) ◽  
pp. 305-313 ◽  
Author(s):  
Cynthia Levy ◽  
Eric Martin
Keyword(s):  
Liver Transplantation ◽  
Liver Disease ◽  
Primary Sclerosing Cholangitis ◽  
Graft Survival ◽  
Sclerosing Cholangitis ◽  
Cholestatic Liver Disease ◽  
Biliary Cirrhosis ◽  
Immune Mediated ◽  
End Stage ◽  
Patient And Graft Survival

AbstractPrimary sclerosing cholangitis (PSC) is a chronic, immune-mediated cholestatic liver disease that often progresses to secondary biliary cirrhosis and end-stage liver disease. Short of liver transplantation (LT), there is no effective treatment for PSC. PSC accounts for approximately 5% of total adult LTs in the US and is currently the fifth most common indication for LT. Patient and graft survival for PSC is among the highest for all indications for LT. The main factors that impact outcomes after LT for PSC include biliary strictures, rejection, and recurrence of PSC. Recurrent PSC (rPSC) develops in 20% of LT recipients within 5 years of LT and is associated with negative patient and graft survival. LT is a viable option for recipients who develop rPSC and progress to graft failure.

Human leukocyte antigen associations in Finnish liver transplantations due to primary sclerosing cholangitis and primary biliary cirrhosis

Open Medicine ◽  
2007 ◽  
Vol 2 (1) ◽  
pp. 12-25
Author(s):  
Irma Matinlauri ◽  
Markku Nurminen ◽  
Krister Höckerstedt ◽  
Helena Isoniemi
Keyword(s):  
Liver Disease ◽  
Primary Biliary Cirrhosis ◽  
Human Leukocyte Antigen ◽  
Primary Sclerosing Cholangitis ◽  
Sclerosing Cholangitis ◽  
Human Leukocyte ◽  
Positive Association ◽  
Biliary Cirrhosis ◽  
Leukocyte Antigen ◽  
Hla Dr3

AbstractA genetic predisposition has been suggested in primary sclerosing cholangitis (PSC) and primary biliary cirrhosis (PBC). The aim of the study was to evaluate human leukocyte antigen (HLA) frequencies and HLA associations in Finnish PSC and PBC patients. The relative frequencies of HLA-A,-B, and-DR antigens were compared between patients with PSC (n=50), or PBC (n=89), transplanted due to end-stage liver disease, and healthy members in the Finnish bone marrow donor registry (n=10000). Prevalence differences, prevalence ratios and the associated large-sample significance probabilities (2-sided P-values) and 95% confidence intervals were calculated.We found a strong positive association between PSC and HLA-B8 and-DR3, and a weak positive association between HLA-A1 and PSC. HLA-DR3 also had a weak positive association with PBC, and a weak negative association between HLA-DR5 and PBC was found. In conclusion, HLA-B8, and-DR3 are susceptible for progressive liver disease in PSC, and HLA-DR3 may also be susceptible for disease progression in PBC. HLA-DR5 may be protective against severe PBC.

Sign in / Sign up

Export Citation Format

Share Document