Re: NEWBIE WITH PSC - LOTS OF QUESTIONS

[Guest guest]

Hi ,

Welcome to the group. I'll try to answer you questions. NEWBIE WITH PSC - LOTS OF QUESTIONS

Hi All,I just found out that I probably have PSC. I haven't had the ERCP yet but the doctors are 95% sure this is what I have.

First off, no one is going to be sure you have PSC until they get the ERCP. I was misdiagnosed for quite a while. I was told I had autoimmune hepatitis with overlap cholestatic liver disease. I would definitely wait for the ERCP before I invested too much effort in PSC. When are they going to do the ERCP? The doctor waited another 4 months in which time I was on Prednisone, which does not help PSC and then I had tremendous problems weaning off of the prednisone.

found that the smooth muscle antibody was detected at 1/20. The smooth muscle antibody is not an indicator for PSC.

She said because of my history with UC and my blood work that I probably have PSC

1) How long does this disease take to progress before my liver will start to fail? There are estimates that say 12 years from dx to tx. But, that is quite vague. Where you have no symptoms, you might guess that you would have more time. We have a member that has lived with PSC for 30 years. Those of us who had symptoms for years and were finally dx, are seldom as shocked as those who discover the disease through routine blood work. No one can predict how long it will take for someone's liver fails.2) Are some cases of PSC milder than others? Yes, PSC can manifest with many different symptoms and those symptoms vary in severity even within the same person. Some of us Know what triggers certain symptoms and try to avoid those triggers.3) Do all cases of PSC ultimately lead to liver failureWell, that is the theory. But, I have a friend who was dx at 59 and just might die of old age before her liver gives out. She is now 72.4) If I have a liver transplant will the new liver ultimately be destroyed by PSC and if so how long will it take?I believe there are varying numbers about that. It does not have the recurrence rate that Hep. C has. We have several members who have had two transplants because PSC returned. Again the time table is very individual. The current rate of recurrence is less than 20% I believe but I'm not sure. 5) What chance is there that PSC could give me bile duct cancer?Yes, this is always a risk. The longer a person has PSC, the higher the risk becomes. Walter Payton died of cholangiocarcinoma before he was able to be transplanted. I don't remember the statistics on how much more at risk we are. 6) Can I have a long healthy productive life (I'm only 25) or am I doomed to kick the bucket in a few years? Yes. We have people who are very productive both pre and post transplant. Right now you are asymptomatic. Eat a low fat, low salt diet. Keep physically active, take calcium, and whatever drugs your doctor recommends. 7) Is there a chance that I will pass this on to my kids? Not very likely8) Do you think there will be new medical advancements in the next decade that will help people with PSC?

Yes, they are working on all kinds of things both pre-tx and post-tx. 9) How long have some of you had PSC and what has it been like?I was dx three years ago, but believe it started about 19 years ago with what they determined was a gall bladder attack. About 9 year later I had my gall bladder out. It had adhesions all over and all through it. I began having horrible episodes of Upper Right Quatraint pain (URQ) that eventually led to a dx of non specific non viral chronic hepatitis. I was never really healthy after I had my gall bladder out. I made many trips to the ER for the upper abdomenal pain. I was checked for lupus, rheumatoid arthritic, everything. I had constant diarrhea, severe itching, weight loss, edema, joint and muscle pain, unbelievable night sweats, increasing fatigue. I became jaundice and they finally checked my liver again. Everyone has stories about how he or she was dx. I quit working last year. I recieve State Disability and will shortly be getting my first Social Security Disability check.

Take care, let us know how the ERCP goes.

Cheryl Berg, Idaho 45Married 25 years, 4 children, 2 grchildrenPSC 01, UC 00, Fibromyalgia, hypothyroid,hiatal hernia, ulcer, gall baldder removed '93disability (retired English teacher)

[Guest guest]

Hi ;

Welcome to the group ... I am very sorry that your health brings you

here, but glad that you found the group.

Others have already answered many of your questions. I would just

simply like to elaborate on a few points. The finding that you are

positive for " smooth muscle antibodies " does not necessarily mean

that you have PSC. These antibodies are typically associated with

autoimmune hepatitis (AIH), although there are cases of " overlap

syndromes " where patients may have both AIH and PSC (or AIH and PBC)

antibodies. Therefore I would agree with what others have said

already, that the results of the ERCP will be very important in your

diagnosis.

Here's a paper on the use of antibodies to distinguish between AIH

and " overlap syndromes " :

________________________

Schweiz Rundsch Med Prax. 2002 Aug 21;91(34):1339-46.

Autoimmune hepatitis and overlap syndrome: diagnosis.

Berg PA, Klein R

Medizinische Klinik Abt. II, Universitat Tubingen.

Autoimmune hepatitis (AIH) is a rare autoimmune disease (incidence

about 5% among all chronic liver disorders) that reflects a loss of

tolerance to normal hepatic proteins. AIH is characterized by female

preponderance, hypergammaglobulinemia, extrahepatic syndromes and a

good response to immunosuppressive treatment. AIH may be subdivided

into two or three subtypes. AIH type 1 is characterized by

antinuclear autoantibodies (ANA) and/or smooth muscle antibodies

(SMA). SMA are actin-specific, can occur without ANA and their

presence relates strongly to AIH. AIH type 2 is defined by the

presence of anti-liver-kidney microsomal antibodies (LKM-1). Patients

with AIH type 2 are typically younger at the time of disease onset,

exhibit higher inflammatory activity, suffer more frequent relapses

under immunosuppressive treatment and are more likely to progress to

cirrhosis. AIH type 3 is characterized by autoantibodies against the

soluble liver antigen (SLA) and liver-pancreas antigen (LP), but

ANA/SMA are frequently present and, therefore, some authors consider

this autoantibody manifestation as belonging to AIH type 1.

Antineutrophil cytoplasmic antibodies (ANCA) recognize cytoplasmic or

nuclear components of neutrophilic granulocytes and are detected with

high prevalence in patients with autoimmune liver diseases. They are

associated with AIH type 1 but not with AIH type 2. However, 40-70%

of patients with primary sclerosing cholangitis (PSC) also produce

these autoantibodies. Autoimmune cholangitis is an idiopathic

disorder with mixed hepatocellular and cholestatic findings that

typically has antinuclear antibodies (ANA). It may be considered as

an atypical form of primary biliary cirrhosis. It has been recognized

that some forms of AIH may also occur with variable incidence and

severity especially in patients with primary biliary cirrhosis

(overlap AIH/PBC) or primary sclerosing cholangitis (AIH/PSC). On the

basis of clinical, biochemical, serological, histological and

radiological criteria a clear distinction between these conditions

can be readily made in the majority of cases. An association of AIH-

typical autoantibodies (anti-LKM-1, anti-SLA/LP) in association with

antimitochondrial autoantibodies (AMA) almost confirm the overlap

syndrome AIH/PBC. In PSC patients expressing typical ERCP findings

and suffering from inflammatory bowel disease (IBD), the diagnosis of

an overlap syndrome between PSC/AIH can be readily made in the

presence of ANCA and AIH relevant autoantibodies. Apart from this

kind of overlap syndrome involving different types of autoimmune

disorders within the liver AIH can be also associated with other

organspecific autoimmune disorders as documented in the autoimmune

polyglandular syndrome type 1 (APS-1). In this disease homozygosity

for a defect in a single gene (AIRE) leads to a broad spectrum of

organ specific autoimmune diseases.

Publication Types:

Review

Review, Tutorial

PMID: 12233264

______________________

To try to paraphrase this paper for you, it says that smooth muscle

antibodies (SMA) are characteristic of type 1 autoimmune hepatitis,

but other antibody tests and ERCP are required to be sure and to rule

out or confirm overlap between AIH and PSC. It might be good to talk

to your doctor about some of these other antibody tests that could be

done to help in your diagnosis.

Why is it important to distinguish between autoimmune hepatitis and

PSC? The strongest reason (and this has already been mentioned by

others) is that autoimmune hepatitis is typically treated with

immunosuppressive therapy, but this does little to help in PSC. It is

possible that autoimmune hepatitis can eventually evolve into PSC, in

which case the AIH becomes resistant to immunosuppression; see the

following article:

____________________________

Hepatology. 2002 Dec;36(6):1393-9.

Evolution of autoimmune hepatitis to primary sclerosing cholangitis:

A sequential syndrome.

Abdo AA, Bain VG, Kichian K, Lee SS

Liver Unit, Division of Gastroenterology, University of Calgary,

Calgary, Alberta, Canada.

Recently, the autoimmune hepatitis (AIH)/primary sclerosing

cholangitis (PSC) overlap syndrome has been reported increasingly. In

this syndrome, patients present with features of both AIH and PSC. It

has been suggested that the 2 diseases may be sequential in their

occurrence, whereby patients have features of AIH and then after a

number of years develop features of PSC, but clear confirmation of

evolution has not been documented in adults. We describe 6 adult

cases in which PSC was diagnosed many years after well-established

AIH. Six patients are described in whom AIH definitely was diagnosed

at presentation. No evidence of biliary disease was noted on the

initial liver biopsy or endoscopic retrograde cholangiography (ERCP).

All patients responded well to immunosuppressive therapy. After an

average duration of follow-up of 4.6 years they became resistant to

immunosuppression, and developed clear features of PSC, which was

confirmed by ERCP in all patients. The average age of the patients at

first presentation was 31.3 years, 2 were women and 4 were men, and 3

had ulcerative colitis. We found no specific features at presentation

that could predict this evolutionary outcome. In conclusion, patients

with well-established AIH can, after variable duration of follow-up,

develop PSC. In patients with AIH who become resistant to

immunosuppression or develop significant cholestasis, PSC should be

ruled out by ERCP.

Publication Types:

Case Reports

PMID: 12447864

__________________________________

So I would definitely wait until the results of the ERCP to be sure

that you have been diagnosed correctly.

One last comment and that would be to remind your doctor to give you

antibiotics following the ERCP, as this could be important in

protecting you against an infection of the bile ducts and/or pancreas.

I think I know how you must be feeling, as my son was 18 when he was

diagnosed with PSC and UC last summer. Try to keep your spirits up,

and keep asking questions!

All the best, and take care,

Dave

(father of (18 yr); dx PSC 07/03; dx UC 08/03)

[Guest guest]

Hi! Welcome to the group :-)

I'll take a shot at your questions here... I'm sure others will have

more info on many of them!

>

> 1) How long does this disease take to progress before my liver will

> start to fail?

This varies widely... I was diagnosed in '92 (at the age of 22) so I've

had it for over 12 years (I had symptoms for a couple years at least

before diagnosis). It sounds like your in the early stages (if what you

have is confirmed to be PSC) so there you could easily have quite a few

years before transplant. Of course it progresses more quickly in some

people...

>

> 2) Are some cases of PSC milder than others?

> 3) Do all cases of PSC ultimately lead to liver failure

I'll take these two together... Theoretically PSC always leads to liver

failure, although in some people the progression is so slow that they

end up dying of other causes before their liver fails. Also, PSC can be

milder in some people in that some people tend to have fewer

complications (some possible ones are infections in the bile ducts and

GI bleeds).

>

> 4) If I have a liver transplant will the new liver ultimately be

> destroyed by PSC and if so how long will it take?

PSC does reoccur sometimes (I've seen numbers as high as 30% reoccurence

rate) but it's certainly not sure to. There are some people here who've

had transplants and now have PSC again, but in any case it does take a

while for the new liver to be damaged (not sure what the range on this

is, but it varies just like PSC in the original liver does)

>

> 5) What chance is there that PSC could give me bile duct cancer?

I've seen studies that list numbers in the 15% range, some higher and

some lower. The longer you have PSC the greater the risk. Smoking and

alcohol have been found (in some studies) to increase the risk, so those

are good things to avoid. I also try to be sure to get plenty of

antioxidants in my diet, and I take Milk Thistle, which has a lot of

antioxidants and is especially active in the liver (although there

haven't been any studies that I know of on it dealing specifically with

bile duct cancer).

> 6) Can I have a long healthy productive life (I'm only 25) or am I

> doomed to kick the bucket in a few years?

Like I mentioned I was diagnosed when I was 22, and now I'm 34. I have

two kids (the youngest is 18 months) and my PSC is pretty stable. I do

have significant liver damage, but for the most part my liver is able to

do it's job although I have to take it easier than I used to.

So I guess the answer to your question is yes, you can live a long

healthy productive life, and you're not doomed to kick the bucket, but

like with everyone else, nothing is assured and you do have an serious

health problem to deal with. It will impact your life, but it doesn't

have to take it away from you.

> 7) Is there a chance that I will pass this on to my kids?

Yes, but very small. Maybe. Actually they don't really know, but there

is a lot of evidence that it does run in families. The thing is that

it's such a rare disease that even if your kids have triple the risk

(to pick something out of thin air) of getting PSC, their risk is still

very low. Like I mentioned I've had two kids since being diagnosed...

to me the small risk is worth the reward!

>

> 8) Do you think there will be new medical advancements in the next

> decade that will help people with PSC?

There are a lot of things being worked on right now. From Tissue

engineering to grow new organs, to new ways of fighting autoimmune

disease, to " liver dialysis " machines. It's hard to tell for sure which

will be successful and in just what way they might help us, but I think

that the chance are good.

>

> 9) How long have some of you had PSC and what has it been like?

I guess I've already answered part of this... For the most part I've

been able to live a pretty normal life. Four years ago I had a GI

bleed, and then went on disability three years ago, due to fatigue.

Actually taking disability has allowed me to live much more normally

than I was before... I'm able to get the rest I need and I'm enjoying

watching the kids grow!

Lots of good questions... I hope you get the answers you need, and I

hope the ERCP gives good results!

athan