Re: HLA gene differences between PSC/UC versus UC alone

[Guest guest]

Dave,This backs what a couple of the Dr.'s at the NIH were saying when they weren't certain that we even had a correct name and understanding of the PSC/UC disease in PSC. That perhaps it was it's own different disease process.LeeIt has long been known that PSC is associated with certain genetic variants of the major histocompatibility complex human leukocyte antigen (HLA) class II region. But since UC susceptibility is also associated with genetic variants in this region, it's not known whether the PSC gene variants are distinct from those associated with UC alone. This study concludes that they are different.

[Guest guest]

Just curious, since I can't remember, but is there a different progression etc...between psc/uc and just plain old psc.

Joan--Mom of 20 psc asymptomatic

-----Original Message-----From: [mailto: ]On Behalf Of leedeubertSent: Friday, February 16, 2007 12:12 PMTo: Subject: Re: HLA gene differences between PSC/UC versus UC alone

Dave,

This backs what a couple of the Dr.'s at the NIH were saying when they weren't certain that we even had a correct name and understanding of the PSC/UC disease in PSC.

That perhaps it was it's own different disease process.

Lee

It has long been known that PSC is associated with certain genetic variants of the major histocompatibility complex human leukocyte antigen (HLA) class II region. But since UC susceptibility is also associated with genetic variants in this region, it's not known whether the PSC gene variants are distinct from those associated with UC alone. This study concludes that they are different.

[Guest guest]

Hi Joan;

I haven't been able to find much evidence that PSC progresses

differently in PSC patients with and without IBD. This was most

recently discussed by Broome and Bergquist:

Broome U, Bergquist A. Primary sclerosing cholangitis, inflammatory

bowel disease, and colon cancer. Semin Liver Dis. 2006 Feb;26(1):31-

41.

http://home.insightbb.com/~rhodesdavid/BroomeU2006.pdf

Comparisons between PSC Patients with and without IBD

" In a study of liver biopsies and autopsy specimens from 43 PSC

patients with and without UC and 19 UC patients with abnormal liver

function tests but normal cholangiograms, Ludwig et al33 found

essentially the same morphological features when PSC was or was not

associated with UC. MacCarty34 et al could not find any differences

between radiological features in PSC patients with and without IBD.

However, Rabinovitz et al,35 who investigated patients with advanced

PSC, found that there were significant differences between PSC

patients with and without IBD. Patients with PSC and IBD were more

likely to be male and to present with abnormal liver function as the

first manifestation of the liver disease, whereas jaundice, pruritus,

and fatigue were the primary symptoms in those patients without

IBD. This finding may well reflect the fact that most UC patients

today are screened regularly using liver function tests, and PSC in

patients with IBD is therefore diagnosed before the onset of liver-

related symptoms. Furthermore, Rabinovitz et al35 showed that the

location of the bile duct involvement in PSC differed significantly

between the two groups. Combined intrahepatic and extrahepatic bile

duct strictures were found more frequently in patients with IBD than

in those without IBD (82 versus 46%). In contrast, involvement of the

extrahepatic bile ducts alone was more frequent in patients without

IBD (38 versus 7%). Among 305 Swedish PSC patients, no difference

between patients with and without IBD could be found.10 At present,

we have insufficient data to conclude that PSC occurring in patients

without IBD is an entity separate from PSC found in association with

IBD. "

Best regards,

Dave

(father of (21); PSC 07/03; UC 08/03)

>

> Just curious, since I can't remember, but is there a different

progression etc...between psc/uc and just plain old psc.

> Joan--Mom of 20 psc asymptomatic

>