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

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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).


Introduction

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.


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