slide1
Download
Skip this Video
Download Presentation
Introduction:

Loading in 2 Seconds...

play fullscreen
1 / 30

Introduction: - PowerPoint PPT Presentation


  • 47 Views
  • Uploaded on

Introduction:. It is the classic hepatobiliary manifestation of IBS. It is generally chronic progressive.

loader
I am the owner, or an agent authorized to act on behalf of the owner, of the copyrighted work described.
capcha
Download Presentation

PowerPoint Slideshow about ' Introduction:' - zamir


An Image/Link below is provided (as is) to download presentation

Download Policy: Content on the Website is provided to you AS IS for your information and personal use and may not be sold / licensed / shared on other websites without getting consent from its author.While downloading, if for some reason you are not able to download a presentation, the publisher may have deleted the file from their server.


- - - - - - - - - - - - - - - - - - - - - - - - - - E N D - - - - - - - - - - - - - - - - - - - - - - - - - -
Presentation Transcript
introduction
Introduction:
  • It is the classic hepatobiliary manifestation of IBS.
  • It is generally chronic progressive.
  • Frequently present with asymptomatic, anicteric cholestasis, but many develop progressive biliary strictures with time, leading to recurrent cholangitis, biliary cirrhosis&end-stage liver disease.
  • Medical treatment does not slow the progression & many patients need liver transplantation, after which recurrent disease is a risk.
  • The increased incidence of hepatobiliary cancer, not related to the underlying severity of biliary fibrosis, is of particular concern.
  • Risk of colorectal cancer is also increased in patients with coexistent IBD.
  • Treatment of is confined to supportive measures
  • Advances in pathobiology suggest that new stratified approaches will soon be available.
epidemiology
Epidemiology:
  • The incidence varies geographically & is as high as 1.3 /100 000 people / year in northern Europe,Prevalence varies 16.2 /100 000.
  • Affects both sexes and all age groups>60% of patients are men.
  • The median age at onset is 30–40 years, usually non-smokers.
  • 60–80% has IBD (30–50% in S Europe & Asia), 75% UC, 83% with UC had a pancolitis, 13% a leftsided colitis, 4% proctitisonly, 95% of those with CD had an ileocolitis & 5% ileitis only.
  • Patients with coexistant IBD are more likely to be male& asymptomatic at presentation&haveboth intrahepatic/ extrahepaticbile duct strictures than are those with PSC only.
  • The prevalence of PSC in colitis varies, but not >10%.
  • CD compared with UC,morelikely to be women,havesmall bile duct disease &a less severe course.
  • Clinically progressive disease has been associated with mild ulcerative colitis& a reduced need for colectomy.
epidemiology1
Epidemiology:
  • PSC without colitis : colitis or immune activation in the gut might simply be too mild to be diagnosed by available techniques.
  • >50% need liver transplantation within 10–15 years of symptom development, as a result of reduced quality and quantity of life related to biliary obstruction, cholangitis, secondary biliary cirrhosis, & hepatobiliary malignant disease.
strictures
Strictures:
  • 10–20% have dominant strictures—ie, stenosis of 1.5 mm or less in CBD or of 1 mm or less in the hepatic duct—many of whom have recurrent bacterial cholangitis.
  • Patients with dominant strictures have significantly worse survival than do those without dominant strictures.
  • Although most dominant strictures are benign, as much as 25% could be malignant.
  • Some patients present with cholelithiasis, gallbladder carcinoma, pancreatitis, or colorectal cancer.
  • PSC is an independent risk factor for CRC in IBD (*4 increased risk)& cumulative risk increases with disease duration (risk of CRC or dysplasia of 9%, 31%, 50% after 10, 20, 25 years, respectively, in patients with PSC a& UC vs2%, 5%, and 10% in those with UC only).
slide6
MRCP:
  • MRCP is the diagnostic test of choice, alongside ultrasonography to exclude secondary causes & assess the gallbladder.
  • MRI showed a sensitivity of 0.86&specificity of 0.94.
  • Strictures, dilatations,pruningof bile ducts are present in both the intrahepatic&extrahepaticbile ducts of roughly 75% of patients&disease is extrahepatic in 5% of patients.
s econdary causes
Secondary causes :
  • Secondary or radiological mimics:
  • Biliary calculi. Cholangiocarcinoma. Biliary tract surgery.
  • Caroli’s dis ease
  • Chronic biliary infection
  • Biliary toxin exposure
  • Chronic portal-vein thrombosis
  • Ischaemicstricturing
  • Cholestaticdrug-induced liver injury
  • Cholangiopathyof critical care.
  • Autoimmune pancreatitis.
  • Slight increases in IgG4 noted in >10% in the absence of clear evidence of AIP & these have a progressive disease phenotype & have no consistent benefits from treatment.
malignancy
Malignancy:
  • PSC associated with an increased risk of cholangiocarcinoma, GB cancer& CRC (in patients with colitis).
  • Advanced fibrosis or cirrhosis are at increased risk of HCC.
  • 2/3 CC are diagnosed at the same time as, or within the first year after, diagnosis of PSC , yearly incidence of is 0.5–1.5%&lifetime risk is at least 10–15%.
  • CC can occur as an intrahepatic mass or a hilartumour.
  • Benign & malignant disease are difficult to distinguish&even the combination of tumour markers, various imaging modalities (MRI, CT, endoscopic ultrasonography) biliary brush cytology (including cytogenetic testing when available) cannot ensure early diagnosis.
malignancy1
Malignancy:
  • High-grade dysplasia on brush cytology has high sensitivity, specificity&positive predictive value for diagnosis, and, when combined with carbohydrate antigen 19-9, sensitivity increases further.
  • Brush cytology necessitates ERCP or PTC which are associated with intrinsic risks.
  • EUS with FNA has greater sensitivity / specificity for the diagnosis of distal CC than do ERCP& brush cytology.
  • Fluorescence in-situ hybridisation, in which DNA probes are used to identify chromosomal changes& digital image analysis, are novel techniques to measure DNA proliferation that might im prove the diagnostic yield of cytology.
  • Other: cholangioscopy-guided biopsies& intra ductal E/S.
  • Urine bio markers by capillary electrophoresis mass spec trometry has generated a CC-specific peptide marker .
malignancy2
Malignancy:
  • Gallbladder cancer develops in 2% &> 50% of gallbladder polyps detected by ultrasonography could be malignant.
  • Thus yearly ultrasonography &a low threshold for cholecystectomy is recommended, but ?true cost-effectiveness unclear.
bo5s 1
BO5s:1
  • Primary sclerosing cholangitis is characterized by all except:
  • A. Usually present with asymptomatic anicteric cholestasis.
  • B. Medical treatments can slow the progression of the disease.
  • C. The treatment of advanced disease is liver transplantation.
  • D. There is risk of recurrence after liver transplantation.
  • E. Risk of CRC is only in those with coexistent IBD.
bo5s 11
BO5s:1
  • Primary sclerosing cholangitis is characterized by all except:
  • A. Usually present with asymptomatic anicteric cholestasis.
  • B. Medical treatments can slow the progression of the disease.
  • C. The treatment of advanced disease is liver transplantation.
  • D. There is risk of recurrence after liver transplantation.
  • E. Risk of CRC is only in those with coexistent IBD.
bo5s 2
BO5s:2
  • Primary sclerosing cholangitis in contrast with primary biliary cirrhosis is charcterized by:
  • A. Affecting more males.
  • B. Affecting younger ages.
  • C. Associated with IBD.
  • D. Associated with autoimmune pancreatitis.
  • E. Responds to UDCA.
bo5s 21
BO5s:2
  • Primary sclerosing cholangitis in contrast with primary biliary cirrhosis is charcterized by:
  • A. Affecting more males.
  • B. Affecting younger ages.
  • C. Associated with IBD.
  • D. Associated with autoimmune pancreatitis.
  • E. Responds to UDCA.
bo5s 3
BO5s:3
  • Primary sclerosing cholangitis associated with UC compared with that associated with Crohn’s disease is characterized by all except:
  • A. More severe course.
  • B. More diffuse disease.
  • C. Affecting more women.
  • D. More diffuse colonic disease.
  • E. Affects more females.
bo5s 31
BO5s:3
  • Primary sclerosing cholangitis associated with UC compared with that associated with Crohn’s disease is characterized by all except:
  • A. More severe course.
  • B. More diffuse disease.
  • C. Affecting more women.
  • D. More diffuse colonic disease.
  • E. About 10%.
bo5s 4
BO5s:4
  • Primary sclerosing cholangitis is associated with all these cancers except:
  • A. Cholangiocarcinoma.
  • B. Gall bladder cancer.
  • C. HCC.
  • D. Pancreatic cancer.
  • E. CRC.
bo5s 41
BO5s:4
  • Primary sclerosing cholangitis is associated with all these cancers except:
  • A. Cholangiocarcinoma.
  • B. Gall bladder cancer.
  • C. HCC.
  • D. Pancreatic cancer.
  • E. CRC.
bo5s 5
BO5s:5
  • Colitis associated with primary sclerosing cholangitis is characterized by all except:
  • A. Starts earlier in life.
  • B. Starts before PSC.
  • C. More total colitis than localized.
  • D. Less rectal sparing.
  • E. More affecting the right side.
bo5s 51
BO5s:5
  • Colitis associated with primary sclerosing cholangitis is characterized by all except:
  • A. Starts earlier in life.
  • B. Starts before PSC.
  • C. More total colitis than localized.
  • D. Less rectal sparing.
  • E. More affecting the right side.
ad