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Re:Small-duct PSC

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Hi Don;

As Debbie has already mentioned, it is pretty challenging to diagnose

small-duct PSC. Apparently the diagnosis is made based on a normal-

looking ERCP (where only medium and large ducts can be visualized),

but where the patient has elevated liver enzymes consistent with bile-

duct involvement/cholestasis (that is, elevated alkaline phophatase

and gamma-glutamyltranspeptidase), and where no other cause of small-

duct injury can be identified. They would have to rule out PBC (which

mostly affects women), and this is done with an antibody test. Most

PBCers (over 95%) have anti-mitochondrial antibodies. But then, some

PBCers (~5 %) are anti-mitochondrial antibody negative. I believe

that anti-mitochondrial antibody negative PBC is sometimes called

autoimmune cholangitis. Liver biopsy might help distinguish between

these three (PSC, PBC and autoimmune cholangitis), and other

autoimmune liver diseases such as autoimmune hepatitis. Finally, they

would also have to rule out drug-induced small-duct loss. There's a

whole series of " ductopenias " (mostly affecting the small-ducts) that

can result from adverse reactions to various drugs.

Best regards,

Dave

(father of (23); PSC 07/03; UC 08/03)

> That's interesting, Dave. Drs  have never mentioned whether my PSC

is small or large duct, but I believe I had/have the small. It might

account for the fact that I've skipped many of the worst symptoms,

especially the frequent painful cholangitis attacks. Are they able to

tell if one has small or large?

 

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