Primary Sclerosing Cholangitis with Case

00:00 So now let's go on to our next case.

00:03 A 32-year-old man presents to clinic to follow up abnormal liver chemistry test.

00:09 His liver test we checked last month to evaluate a 3-month history of fatigue.

00:14 He has a history of diarrhea for the past nine months but has no known history of liver disease.

00:19 He has not had any abdominal pain, fever or jaundice.

00:23 Vitals are normal.

00:24 His exam reveals hepatomegaly but no splenomegaly.

00:29 There is no ascites, jaundice or spider angiomata.

00:33 Labs are notable for an AST of 92 (U/L), ALT 65 (U/L), alkaline phosphatase 475 (U/L) and total bilirubin of 1.4 (mg/dL) His abdominal ultrasound shows an enlarged liver with thickened extrahepatic bile duct but no intrahepatic biliary dilatation or masses.

00:53 So what is the best next step in diagnosis? Let's point out that he has a combination of fatigue, with abnormal liver test which should increase your suspicion for some type of hepatobiliary disease.

01:07 He does also have a history of chronic diarrhea which may be important a bit later.

01:13 In addition, on his physical exam and labs, he has hepatomegaly and a cholestatic pattern of elevation.

01:20 The ultrasound shows abnormal extrahepatic bile ducts but no other obvious abnormalities of the liver.

01:28 So that’s brings us to the diagnosis of primary sclerosing cholangitis or PSC.

01:33 This is an inflammatory disorder that affects the biliary system which can be either within or outside of the liver.

01:41 It tends to affect men more than women.

01:43 And will manifest with fatigue, a generalized pruritus and jaundice.

01:49 Both of these are caused by increased bilirubiin.

01:52 In addition, it can be associated with complications.

01:55 So the disease may progress and lead to cirrhosis, or scarring of the liver.

02:00 In addition, it is also associated with a high risk of cholangiocarcinoma.

02:05 It is also associated with many autoimmune diseases, in particular, ulcerative colitis.

02:12 And the diagnosis is made by looking for a cholestatic pattern of injury on your liver test, and performing an MRCP or ERCP to confirm the diagnosis.

02:24 Unfortunately for this condition, there is no really effective medical therapy.

02:30 What we can do instead is perform endoscopic dilatation of any strictures that are formed in the biliary system.

02:37 And when the disease becomes very severe, we can refer for liver transplantation.

02:44 So, we mentioned earlier that PSC has certain risks of different types of cancers.

02:49 It increases your risk of cholangiocarcinoma so patients who have this condition have a 10-15% risk in their lifetime of developing this type of cancer.

02:59 They are also at high risk for colon cancer, hepatocellular cancer if their disease progresses to cirrhosis and other types of gallbladder cancer.

03:11 So, earlier we mentioned that you could use an MRCP to make this diagnosis.

03:16 AN MRCP is magnetic resonance cholangiopancreatography Here on the left side you can see an example of normal common bile duct anatomy.

03:26 So you can see a normal common bile duct and normal hepatic ducts arising from it.

03:31 On the right side, this is an example of an MRCP showing the typical "beaded ducts" appearance of PSC so you see a very prominent and irregular looking common bile duct and irregular hepatic ducts as is seen in PSC.

03:47 So now we return to our case.

03:50 A 32- year-old man coming in with fatigue, known abnormal liver tests, chronic diarrhea which potentially raises your suspicion for inflammatory bowel disease such as ulcerative colitis and a cholestatic pattern of elevation with ultrasound showing abnormal extrahepatic bile ducts but no other obvious abnormalities.

04:11 So at this point, you should be suspecting PSC and the best next step to confirm the diagnosis is to do an MRCP or ERCP.

04:22 Thank you very much for your attention.