Primary Sclerosing Cholangitis

by Carlo Raj, MD

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    Primary Sclerosing Cholangitis. Break up the name. Primary beginning, arising by itself. Sclerosing; narrowing of what? The bile duct. That is your topic. Next. What predictors of what we are going to discuss? It is an autoimmune disease for the most part. Here, it is not AMA positive. AMA positivity was found in Primary Biliary Cirrhosis. Here, upon histology, I'd tell you about the onion skinning of the bile duct. And with primary sclerosing cholangitis, If you narrow, one part of the bile duct, May I ask you a question, physiologically, If you take any tube, such a blood vessel or a duct or whatever, If you then narrow the distal end, what happened to the proximal end? Pathologically. Dilation. So you have narrowing. Dilation, narrowing, dilation. Doesn't it sound awfully a lot maybe perhaps Some of you might be wearing necklaces, beaded appearance. When would you notice a beaded appearance with primary sclerosis cholangitis On an imaging study. And we call that a cholengiogram. Let's begin. Inflammatory destruction of medium and large bile ducts; this affects however, young men. More so. 70% are associated with ulcerated cholitis. This is something that you will never find with primary biliary cirrhosis. you will not find this. 70% percent is associated with ulcerated cholitis. Where is that? Rectum. Alright? Remember, the rectum, continuous, ulcers, proximaly so, you move from the rectum up the descending colon and so forth. And because of this, there is an increased risk of colorectal cancer CRC. You see how different PSC is from PBC. Spend a little time to make sure you are clear between the two diagnoses. You cannot afford to get the two confused. Along with colorectal cancer, and ulcerated colitis, there is a major major, association with cholengio carcinoma. So, not only there could...

    About the Lecture

    The lecture Primary Sclerosing Cholangitis by Carlo Raj, MD is from the course Cirrhosis – Liver Diseases.

    Included Quiz Questions

    1. Onion skinning appearance
    2. Absence of bile ducts
    3. Fibrotic bands around the lobules of hepatocytes
    4. Noncaseating granulomas
    5. Lymphocytic infiltration limited to the portal triad only
    1. Colon cancer and cholangiocarcinoma
    2. hepatocellular carcinoma and cholangiocarcinoma
    3. Hepatocellular carcinoma and colon cancer
    4. Pancreatic adenocarcinoma and hepatocellular carcinoma
    5. Pancreatic carcinoma and cholangiocarcinoma
    1. Beaded appearance
    2. Rat tail appearance
    3. Bird beak appearance
    4. double bubble sign
    5. Snow storm sign

    Author of lecture Primary Sclerosing Cholangitis

     Carlo Raj, MD

    Carlo Raj, MD

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