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Primary Sclerosing Cholangitis

sclerosing cholangitis psc primary sclerosing limited scleroderma lssc primary biliary pbc liver involvement liver

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#1 Sierra Sierra

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Posted 24 May 2016 - 11:21 AM

It's been a long, long time since I was last here - busy enjoying life and battling on despite the health problems. 

 

I seem to be making a habit of collecting rare conditions and that's what brings me back.  I recently 'celebrated' my 7th scleroderma birthday (7 years since diagnosis of limited cutaneous systemic sclerosis) and I'm wondering how many of us 'sclerodermians' also have Primary Sclerosing Cholangitis (PSC).  Who better to ask than the expert patients on the forums. 

 

After several years of poor liver function tests they reached a stage where action was needed and I was diagnosed in November 2015 with PSC.  Since then I have done extensive research and educated myself as much as possible.  My understanding is that the expectation for 'sclerodermians' with liver problems is usually Primary Biliary Cirrhosis (PBC), which is what the consultants thought was my problem, but there is now no doubt that my diagnosis is PSC. 

 

I have only managed to find one research paper covering limited cutaneous systemic sclerosis and PSC in the same patient and, apparently, that was the first case known (at least to the paper's authors) and that was in March 2015.  I can't believe that I am only the second person known to have both diseases so thought I'd post this in the hope that there are more of us out there (not that I would wish it on anyone, of course).  If so, I hope to be able to compare notes as neither my scleroderma Prof, nor my hepatobiliary consultant have ever come across both diseases in one patient and are unable to answer any of my questions.  We are all on this magical mystery tour and learning together! 

 

So, if any of you have both limited cutaneous systemic sclerosis and primary sclerosing cholangitis, please let me know so that I can get an idea of numbers.  I'm sure my consultants would be interested to know too.  Thanks in advance.



#2 Joelf

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Posted 25 May 2016 - 06:15 AM

Hi Sierra,

 

How lovely to hear from you again, although I'm sorry that it's because you're experiencing such an unusual and unpleasant complication such as primary sclerosing cholangitis.

 

I must admit that I've never heard of it either, especially in conjunction with scleroderma. I've only found one article Case Report: Primary sclerosing cholangitis associated with CREST in an elderly woman which I've included on our Liver and Spleen Involvement page, which possibly may be the one to which you refer (this one is dated November 2015.)

 

It is possible that we could have other members with this complication, so hopefully you may get some more first hand information about it.

 

Kind regards,


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#3 Sierra Sierra

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Posted 25 May 2016 - 07:30 AM

Hi Joelf and thanks for your speedy response.  Yes, that is the case that I found - it was written around May 2015 and accepted 26th October 2015 but wasn't published until November 2015.  The article does state that PSC has been known with diffuse cutaneous scleroderma but 'has not been previously reported in the literature' in connection with limited.  As I said, I can't believe that I'm only the second person known to have both, although if I am I must really be getting close to being one in a million!!  It will be interesting to see if anyone else replies.

 

Best Wishes

 

Sierra



#4 Shelley Ensz

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Posted 26 May 2016 - 08:19 AM

Hi Sierra,

 

I thought you might be interested in this 2010 article posted in our forums, which is about tumeric (the Indian spice) delaying the progression of primary sclerosing cholangitis, in scleroderma patients.  See: Tumeric Delays Liver Disease Progression

 

I thought it might help for me to point out that everyone in the UK can also post in our Main forum, unless you have a question specific to scleroderma resources/experts in the U.K.  And, everyone in the forums may also post in the UK forums, you do not have to be a UK resident to respond to messages here. Anyway, since this particular message could use larger exposure, to help discover other lSSc/PSC'ers, I'm going to move it into our Main forum.

 

:hug-group:


Warm Hugs,

Shelley Ensz
Founder and President
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Hotline and Donations: 1-800-564-7099

The most important thing in the world to know about scleroderma is sclero.org.

#5 Sierra Sierra

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Posted 27 May 2016 - 09:28 AM

Hi Shelley

 

Many thanks for both the article and moving my post to the Main forum. 

 

I was aware of the benefits of turmeric through my research but hit a snag when several of the articles I read stated that turmeric shouldn't be taken by those with a bile obstruction, which is one of the problems caused by PSC.  I had pharmacists contacting specialist centres and doing their own researching, and the overwhelming advice was not to take it.  However, I don't give up easily (!) and presented my hepatobiliary consultant with all the articles, all the information from pharmacists and all the instances of turmeric being contra-indicated and then persuaded him to let me try it.  Nothing to lose and everything to gain.  So he is now going to monitor my bilirubin levels very closely and if they start to rise I will have to stop taking the turmeric.  Although I have seen an improvement in symptoms since starting on Ursodeoxycholic Acid it apparently won't slow down the progression so I'm hoping that turmeric will help, as well as helping with inflammation generally etc etc.  It's considered by some to be something of a miracle worker - here's hoping!

 

Best wishes.

 

Sierra



#6 missmoneypenny

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Posted 17 September 2016 - 07:09 AM

Hi Sierra, just thought you would be interested to know that I have PSC and had a liver transplant in 1990 due to that condition. I also have CREST diagnosed in 2009 ,so sorry to inform you that you are not the only person to have both! I know that scleroderma is usually found in PBC so we are obviously 2 rare birds. I also have ulcerated colitis and crohn's and now I have been diagnosed with celiac disease. It seems once you have one autoimmune condition you begin to develop more and more. I am also having problems with my digestive system ,obviously the scleroderma is affecting the muscles in the stomach, feeling full after eating very little. The liver transplant was nothing compared to this scleroderma. I'm here if you need any more information regarding PSC or about liver transplantation .

#7 Joelf

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Posted 17 September 2016 - 09:18 AM

Hi Missmoneypenny,

 

Welcome to these forums!

 

I'm sorry to hear that you're suffering with so many autoimmune problems and have had to undergo a liver transplant.

 

I do hope that you are obtaining treatment from a good medical team, as these rare conditions do need specialist help and expertise.

 

Kind regards,


Jo Frowde
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#8 missmoneypenny

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Posted 20 September 2016 - 06:28 AM

Hi Joelf, thanks for your welcoming e-mail.
Yes I have had excellent medical treatment through the NHS all my life from the age of 10 when I was diagnosed with ulcerated colitis and crohns ( I've got to be greedy!) until now -aged 61. If I had been born in another country and not Britain especially countries where you need health insurance I would have died many years ago. My gastroenteritis doctor retired last March I had been seeing him for the last 30 years what a wonderful doctor . I now have a new team and I also have a great rheumatologist for my oesteoarthritis and scleroderma.

#9 Sierra Sierra

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Posted 09 October 2016 - 09:47 AM

Hi Missmoneypenny

Thanks for your reply - as usual I've been busy working (had my first day off in 3 months just this week!!), attending medical appointments and treatments, and enjoying life and so haven't been back to the forum for a while. I'd almost given up hope of finding anyone else with both limited cutaneous systemic sclerosis and PSC. It's a lonely place to be! It brings me no joy, however, to have found another very rare person and I truly sympathise with the battles you must have on a daily basis. I know what it's like! :crying:

From the info you've given it seems that PSC came first for you, whereas scleroderma came first for me. Thankfully I don't have ulcerative colitis (not yet, at any rate) which is again rare as I believe that 70% of PSCers do have it. I too had problems with feeling full for ages after eating very little but that has improved greatly since I've been on Urso. I'm guessing that you're also on Urso (ursodiol)? I'd had a very strange, and horrific, pain for several years which I found very difficult to describe, or pinpoint its exact location when trying to describe it to my doctors. I put it down to the scleroderma related issues of gastritis, oesophagitis, duodenitis, duodenal ulcer, poor peristalsis and hiatus hernia. It wasn't touched by painkillers and on many occasions reduced me to tears. It disappeared as soon as I started on Urso so that has been something of a miracle drug for me - even though it apparently won't slow the disease progression. Do you take, or have you tried, turmeric? I mentioned it in my previous posts and tried it for 3 months. It had no effect on my liver test results and didn't benefit me at all as a general anti-inflammatory. One of my other issues is chronic erythema nodosum (panniculitis) and sclerosing panniculitis and I really hoped the turmeric would help with that but it hasn't. My latest, and current flare-up has been going since March this year and is showing no sign of clearing. I was also diagnosed with Restless Legs Syndrome in August. It never ends, does it?! RLS can be caused by iron deficiency and/or kidney disease and I seem to have both so am currently taking iron tablets (month 2 of a 3 month course). I haven't noticed any improvement so far. My kidney tests are being closely monitored. I wanted to pursue both avenues before starting on yet more meds specifically for RLS.

Do you have any tips for me on dealing with PSC? Has it affected your new liver? Do you have any info as to how PSC affects scleroderma or vice versa? I have so many questions and it seems that you may be the only person in the world who may be able to answer them! :yes: :thank-you:
Take care and be strong x

#10 judyt

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Posted 09 October 2016 - 01:45 PM

Hi Sierra,

You are by no means alone with your plethora of AI problems.   I have had deranged liver function for many years and finally in 2012 it was diagnosed as PBC Primary Biliary Cirrhosis.   Since then I have been on Urso and have little or no obvious disease.   Since then the name for PBC has been changed (by whom exactly I don't really know) to PSC because it has been said that the word Cirrhosis is too stigmatising for those of use with AI disease rather than Alcohol induced disease.   Who knows, as far as I can see PBC and PSC are different things but since I am not a Doctor who am I to say.

 

Anyway you are certainly not alone with all these things plaguing us.   I really do not notice anything in particular which affects me more than it did before, I have reflux, gastritis and bowel incontinence just like so many others do.   Fortunately I don't notice any particular itch which is common with PBC/PSC.   Fatigue is a problem and as well I have blood loss anaemia because of the damage to my bowel.  Apparently Telangiectasia can form in the organs and bleed which has happend for me.

 

Kia Kaha - Be Strong

Best wishes

Judyt



#11 Sierra Sierra

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Posted 10 October 2016 - 10:21 AM

Hi Judyt and thank you for your post.

You are absolutely right when you say that 'PBC and PSC are different things'. The name change of Primary Biliary Cirrhosis to Primary Biliary Cholangitis was 'announced' at the European Association for the Study of the Liver Conference in Vienna in April 2015. At that conference, Professor Ulrich Beuers of the Tytgat Institute for Liver and Intestinal Research commenced his address to the delegates by announcing that it would be the last time he referred to PBC as Primary Biliary Cirrhosis, as from now on it would be called Primary Biliary Cholangitis.

This explains the difference between PSC and PBC

 

Primary Sclerosing Cholangitis: What's in a Name?

It may prove useful to you when dealing with doctors!

I have also had problems with telangiectasias and I recently had to have both sides of my nose cauterised because of the little bleeders!! My nose continued to bleed (but nowhere near as badly) for a few weeks after the procedure but now, fingers crossed, seems to have stopped. My ENT doc said that may happen but then I should be OK until or unless I develop more telangiectasias, in which case I may need further cauterisation.

Anyway, thank you for replying and I love the Kia Kaha. I think I will adopt it into my vocabulary.

Kia Kaha and best wishes.

Sierra x



#12 judyt

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Posted 11 October 2016 - 10:36 AM

Sierra,

 

Thank you for that information, I had never thought of researching the names in that way and it does clarify things very nicely.

 

I have got over being upset about the Alcohol inferences, but now I have to teach myself to use the word Cholangiitis in place of Cirrhosis!!

 

Judyt







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