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Introduction:

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

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Introduction:

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  1. 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.

  2. 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.

  3. 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.

  4. 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).

  5. 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.

  6. 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.

  7. 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.

  8. 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 .

  9. 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.

  10. 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.

  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.

  12. 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.

  13. 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.

  14. 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.

  15. 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%.

  16. 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.

  17. 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.

  18. 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.

  19. 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.

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