Primary Sclerosing Cholangitis

Primary Sclerosing Cholangitis

Q. 1

Primary sclerosing cholangitis, true are ‑

 A T Females

 B Associated with IBD

 C

Involves intra & extrahepatic ducts

 D

b and c

Q. 1

Primary sclerosing cholangitis, true are ‑

 A

T Females

 B

Associated with IBD

 C

Involves intra & extrahepatic ducts

 D

b and c

Ans. D

Explanation:

Ans. is ‘b’ i.e., Associated with IBD; ‘c’ Involves intra & extrahepatic ducts

Primary sclerosing cholangitis (PSC)

o Is a fibrosing cholangitis of bile ducts leading to inflammatory strictures and obliteration of both intrahepatic and extrahepatic ducts with dilatation of preserved segments.

o It’s a progressive disease eventually resulting in secondary biliary cirrhosis.

o It’s of Unknown etiology, association is seen with HLA-B8, -DR3, -DQ2 and – DRw52A

o Its seen to be associated with ulcerative colitis and occasionally Crohn’s disease (Ulcerative colitis seen in approx 2/3″ of patients of PSC).

o Other diseases associated with PSC

             Riedel ‘s thyroiditis

             Retroperitoneal or mediastinal .fibrosis

             Pseudotumor of orbit

o PSC is a risk factor for Cholangiocarcinoma

o Morphology

             “PSC is a fibrosing cholangitis of bile ducts, with a lymphocytic infiltrate, progressive atrophy of the bile duct epithelium and obliteration of the lumen. The concentric periductal fibrosis around the affected ducts (“onion skin fibrosis”) is followed by their disappearance leaving behind a solid cord-like fibrous scar. In between areas of progressive stricture, bile ducts become ectatic and inflamed, presumbly the result of downstream obstruction. As the disease progresses, the liver becomes markedly cholestatic, culminating in biliary cirrhosis much like that seen with primary and secondary biliary cirrhosis”.

o Clinical Picture

         Mean age of presentation is 30 to 45 yrs.

               M : F ratio – 2 : 1

         Usual presentation is intermittent jaundice, fatigue, weight loss, pruritus and abdominal pain.

         Asymptomatic patients may come to attention because of persistent elevation of serum alkaline phosphatase.

         Clinical course is highly variable with cyclic remissions and exacerbations. Some may remain asymptomatic for years, others may progress very rapidly to sec. biliary cirrhosis and liver failure.

o The clinical presentation and increased alkaline phosphatase and bilirubin levels may suggest the diagnosis, but Endoscopic retrograde cholangiography is the definitive investigation.

ERC shows multiple dilatation and strictures involving both intrahepatic and extrahepatic ducts.

         MRCP is the best non-invasive investigation. It is less sensitive than ERCP for intrahepatic ducts.


Q. 2 Primary sclerosing cholangitis is likely to be associated with –

 A Adenocarcinoma of pancreas

 B

Cholangiocarcinoma

 C

Hepatocellular carcinoma

 D

Adenocarcinoma of gall bladder

Ans. B

Explanation:

Ans. is ‘b’ i.e., Cholangiocarcinoma

PSC is a risk factor for cholangiocarcinoma.


Q. 3

True about primary sclerosing cholangitis are all Except

 A

fibrosing cholangitis of bile duct

 B

periductal onion skin appearance

 C

Cirrhosis never occurs

 D

jaundice seen

Ans. C

Explanation:

Answer is ‘c’ i.e. Cirrhosis never occurs

  • Primary sclerosing cholangitis (PSC)
  • is a fibrosing cholangitis of bile ducts leading to inflammatory strictures and obliteration of both intrahepatic and extrahepatic ducts with dilatation of preserved segments.
  • It’s a progressive disease eventually resulting in secondary biliary cirrhosis.
  • Its of Unknown etiology

association is seen with HLA-B8, -DR3, -DQ2 and -DRw52A.

Its seen to be associated with ulcerative colitis and occasionally Crohn’s disease (Ulcerative colitis seen in approx 2/f of patients of PSC)

  • Other diseases associated with PSC

–      Riedel’s thyroiditis

– Retroperitoneal or mediastinal fibrosis

– Pseudotumor of orbit

  • PSC is a risk factor for Cholangiocarcinoma

– 10 to 20% of patients with PSC develop cholangiocarcinoma

– Cholangocarcinoma can present at any time during the disease process and does not correlate with the extent of sclerosing cholangitis.

Morphology

  • Robbins writes – “PSC is a fibrosing cholangitis of bile ducts, with a lymphocytic infiltrate, progressive atrophy of the bile duct epithelium, and obliteration of the lumen. The concentric periductal fibrosis around the affected ducts (“onion skin fibrosis”) is followed by their disappearance leaving behind a solid cord-like fibrous scar. In between areas of progressive stricture, bile ducts become ectatic and inflamed, presumably the result of downstream obstruction. As the disease progresses, the liver becomes markedly cholestatic, culminating in biliary cirrhosis much like that seen with primary and secondary biliary cirrhosis.”
  • Clinical Picture

– Mean age of presentation is 30 to 45 yrs.

– M : F ratio – 2 : 1

Usual presentation is intermittent jaundice, fatigue, weight loss, pruritus and abdominal pain. Asymptomatic patients may come to attention because of persistent elevation of serum alkaline phosphatase.

Clinical course is highly variable with cyclic remissions and exacerbations. some may remain asymptomatic for years, others may progress very rapidly to sec. biliary cirrhosis and liver failure.

  • The clinical presentation and increased alkaline phosphatase and bilirubin levels may suggest the diagnosis, but Endoscopic retrograde cholangiography is the definitive investigation.

ERC shows multiple dilatation and strictures involving both intrahepatic and extrahepatic ducts.

– MRCP is the best non-invasive investigation. It is less sensitive than ERCP for intrahepatic ducts.

  • Treatment

–  there is no known effective medical treatment.

– Liver transplantation is the only option


Q. 4

True regarding primary sclerosing cholangitis associated with ulcerative colitis are all of the following except:                                 

March 2007

 A Biliary Cirrhosis is a known complication

 B Increased risk of hilar cholangiocarcinoma

 C

May have raised levels of alkaline phoshphatase

 D

Primary Sclerosing Cholangitis resolves after total colectomy

Ans. D

Explanation:

Ans. D: Primary Sclerosing Cholangitis resolves after total colectomy

Primary sclerosing cholangitis (PSC) is a form of cholangitis due to an autoimmune reaction. Primary sclerosing cholangitis leads to cholestasis.

Blockage of the bile duct leads to accumulation of bile, which damages the liver, leading to jaundice and eventually causes liver failure.

Signs and symptoms

  • Tiredness (a non-specific symptom often present in liver disease)
  • Severe jaundice with intense itching.
  • Malabsorption (especially of fat) and steatorrhea, leading to decreased levels of the fat-soluble vitamins, A, D, E and K.
  • Signs of cirrhosis
  • Ascending cholangitis‑

The diagnosis is by imaging of the bile duct, usually in the setting of endoscopic retrograde cholangiopancreatography, which shows characteristic changes (“beading”) of the bile ducts.

Another option is magnetic resonance cholangiopancreatography (MRCP).

The differential diagnosis can include primary biliary cirrhosis, drug induced cholestasis, cholangiocarcinoma, and HIV-associated cholangiopathy.

PSC is often listed as a manifestation of ulcerative colitis outside the colon.

PSC differs from these manifestations in that, unlike most other manifestations, PSC continues in spite of surgical removal of the colon.

Standard treatment includes ursodiol.

Treatment also includes medication to relieve itching (antipruritics) and bile acid sequesterants (cholestyramine) etc.


Q. 5 True about primary sclerosing cholangitis:

 A Involves only intrahepatic bile duct, not extrahepatic bile duct

 B

Associated with Inflammatory bowel disease

 C

Causes macronodular cirrhosis

 D

Periductal fibrosis of smaller bile ducts

Ans. B:D

Explanation:

Answer- (B) Associated with Inflammatory bowel disease (D) Periductal fibrosis of smaller bile ducts

  • PSC is characterized by lnflammation and obliterative fibrosis of lntrahepatic and extrahepatic bile ducts wlth dilation of preserved segments.
  • Inflammatory bowel disease , particularly ulcerative colitis, coexists in approximately 70% of individuals with PSC.
  • Following changes are seen-fibrosing cholangitis, periductal fibrosis, dilation of intervening bile ducts and cholestatis with full blown picture of biliary cirrhosis”


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