Re: PSC, PBC, both, or neither (To Dave )

[Guest guest]

Dave,

I am so very gratefull that you replied to my post and gave me all

of that information. Granted, now I'm more anxious now but I think

I know what's going on and it helps me to be prepared for bad news.

I have had the AMA tests done 4 times. The first time it came back

strongly positive. 2 weeks later it came back negative. I then got

in my car and drove to UNMC, a great hospital here in Nebraska. I

went to the ER and by the end of the day I was being seen by Dr

Sorrell. He's been in hepatology fro 35 years and often meets with

Dr Lindor and Mayo in MN and some PBC specialist in Sacramento CA.

He did a bunch of labs and u/s's and stuff. 2 weeks later I came

back and he said my AMA was strongly positive and dx with PBC. I

had just found out I was pregnant so we waited until after delivery

to do the biopsy.

So after that came back good he did the AMA test again (4th one) and

it was negative. So he thinks I may be in early PBC, because of 2

positive AMA and 2 negative. Then they did the MRCP and my common

bile duct is very constricted and the smaller ones are also. I

guess that's why he's thining PSC.

So I guess the only way to finally figure it out is the ERCP and

they may do a stent which should help with the pain, or so I'm

told.

I feel like I have no other choice because I have so many symptoms

and have been dx with so many things just for them to come back and

say...well no you don't have that, hmmmmm. (Lupus, AIH, UC,

lymphoma, PBC...not to mention the doctors that though I was just a

drug seeker) Thank you for that information. It was very helpful.

My ERCP is scheduled for next Thursday so we'll see what they say

then.

>

> Hi ;

>

> At your initial diagnosis of PBC, were any antibody tests done?

> Antibody tests, using blood samples, can be very helpful in

> distinguishing between various types of autoimmune liver diseases.

> PBC is characterized by anti-mitochondrial antibodies:

> ___________________________

>

> Mod Pathol. 2007 Feb;20 Suppl 1:S15-30.

>

> Autoimmune liver disease: overlap and outliers.

>

> Washington MK

>

> Department of Pathology, Vanderbilt University Medical Center,

> Nashville, TN, USA. kay.washington@...

>

> The three main categories of autoimmune liver disease are

autoimmune

> hepatitis (AIH), primary biliary cirrhosis (PBC), and primary

> sclerosing cholangitis (PSC); all are well-defined entities with

> diagnosis based upon a constellation of clinical, serologic, and

> liver pathology findings. Although these diseases are considered

> autoimmune in nature, the etiology and possible environmental

> triggers of each remain obscure. The characteristic morphologic

> patterns of injury are a chronic hepatitis pattern of injury with

> prominent plasma cells in AIH, destruction of small intrahepatic

bile

> ducts and canals of Hering in PBC, and periductal fibrosis and

> inflammation involving larger bile ducts with variable small duct

> damage in PSC. Serological findings include the presence of

> antimitochondrial antibodies in PBC, antinuclear, anti-smooth

muscle,

> and anti-LKM antibodies in AIH, and pANCA in PSC. Although most

cases

> of autoimmune liver disease fit readily into one of these three

> categories, overlap syndromes (primarily of AIH with PBC or PSC)

may

> comprise up to 10% of cases, and variant syndromes such as

> antimitochondrial antibody-negative PBC also occur. Sequential

> syndromes with transition from one form of autoimmune liver

disease

> to another are rare. PMID: 17486048.

> _____________________

>

> As indicated, anti-smooth muscle, and anti-LKM (liver/kidney

> microsome) antibodies occur in AIH, and pANCA (peri-nuclear anti-

> neutrophil cytoplasmic antibodies) in PSC. It would seem to me

that a

> firm initial diagnosis of PBC would have to include a positive

anti-

> mitochondial antibody (AMA) test [however, admittedly a small

> fraction of PBC patients are anti-AMA negative, and I believe that

> this variant is sometimes called autoimmune cholangitis!]. PBC

> occurs mostly in women, and mostly affects the small bile ducts.

>

> If the MRCP is now showing damage to the large ducts consistent

with

> PSC, I might recommend having the MRCP film reviewed by someone

who

> is an expert in PSC diagnosis (e.g. hepatologists at Mayo clinic).

By

> looking at the film, they may be able to tell you whether an ERCP

is

> necessary or not. Complications can occur as a result of ERCPs,

and

> you should be aware of these complications before having one done.

> Research studies suggest that the fewest complications occur when

the

> ERCPs are done by expert endoscopists!

>

> I have only come across one report where PBC was initially

diagnosed,

> and the patient then later developed PSC:

> __________________

>

> Dig Dis Sci. 2001 Sep;46(9):2043-7.

>

> A case of coexisting primary biliary cirrhosis and primary

sclerosing

> cholangitis: a new overlap of autoimmune liver diseases.

>

> Burak KW, Urbanski SJ, Swain MG

>

> Liver Unit, University of Calgary, Alberta, Canada.

>

> Although the etiology of AIH, PBC, and PSC remains unknown, it is

> apparent that these autoimmune liver diseases share many common

> features and can coexist in the same patient. Our patient had

> features of PBC and later clearly developed a picture of PSC. This

> case suggests that PBC, PSC, AIH, and autoimmune cholangitis are

part

> of a spectrum of chronic autoimmune liver disease that develop in

> response to some yet unidentified antigen. PMID: 11575461.

>

> __________________________

>

> I hope that you get a firm answer soon.

>

> Best regards,

>

> Dave

> (father of (22); PSC 07/03; UC 08/03)

>

[Guest guest]

Dear ;

I am sorry if my comments made you more anxious. I was just concerned

that if your doctors were planning to do an ERCP for diagnostic

purposes, it might not tell much more than the results of the MRCP. It

sounds like they plan to do a therepeutic ERCP to open up a bile duct

stricture, and I guess there is no substitute for that. It sounds like

you are in good hands ... I am glad to hear that your hepatoligist is

in close contact with Dr. Lindor at Mayo clinic. The different AMA-

positive and AMA-negative results are puzzling but it seems like you

are finally getting some answers. I wish you all the best with the ERCP.

Best regards,

Dave R.