Step I Primary Biliary Cirrhosis

[blackbird03]

help! if it is due to an autoimmune disorder in which there is sclerosing of the bile ducts, does it often present with jaundice? why or why not?

 

[2TIM4:7]

help! if it is due to an autoimmune disorder in which there is sclerosing of the bile ducts, does it often present with jaundice? why or why not?

it's not due to sclerosing but an autoimmune attack by mononuclear infiltrates (granulomatous reaction)..robbins has a good picture (primary sclerosing cholangitis has sclerosis--onion skinning)

primary biliary is a slowly progressive disease and may present with jaundice later...

 

[blackbird03]

Thanks very much TIM,

I dont' doubt the veracity of your reply, however, qbank says this:

"The patient's disease is primary biliary cirrhosis, which is an autoimmune disease characterized by sclerosing cholangitis and cholangiolitis. The most helpful clue in the question stem is the antimitochondrial antibody, which is present in over 90% of the patients. The course is usually slowly progressive over 5-25 years. Patients are sometimes diagnosed when routine blood tests demonstrate an elevated alkaline phosphatase. In addition to the features listed in the question, patients with advanced disease tend to develop profound hypercholesterolemia and xanthomas."

Does PBC always arise from PSC? Is sclerosing always present in PBC as qbank assumes? I guess the qbank answer confuses me...

 

[2TIM4:7]

Hmmm...this is something i've found out (as well as you probably)...KAPLAN is WRONG not all of the time, but frequently enough! 🙂

I don't know what kaplan is smoking but if PSC converts to PBC then there is a problem with disease categorization...PBC is associated with a lot of autoimmune diseases such as celiac, sjogren, rheumatoid, etc. PSC is associated with UC.

Just today Kaplan told me that FEV1 in restrictive fibrotic lungs is normal!!! and that a 19 y.o. with meningitis is infected most probably with S. pneumonia!!! 🙄