Saying hello and some questions

Welcome New Member Introductions
1

Hi everyone!

I’ve been reading on this site quite a lot and it’s wonderful to see how you guys help each other out! Finally I decided to become a member as well, also because I have some questions regarding psc.

Some background information:
I’m Sophie, 26 years old from the Netherlands. I’ve been diagnosed with Crohn’s disease when I was 13 years old and had a lot of flare ups causing endless medications, treatments and surgeries. Thankfully I’ve also had some pretty good times! Currently, I’m on Vedoluzimab (Entyvio).
PSC was diagnosed after high liver enzymes in blood work (they tested me for it because I was using methotrexate). I had to stop methotrexate but my liver enzymes only elevated. That’s how I became diagnosed with psc in December 2014 after an MRCP.

Until October 2016 I didn’t really have any symptoms regarding psc and I tried (keyword tried) not to look up too much about psc. I tried to “ignore” it, but around this october I started to feel less energetic, more diarrhea (also back pain and headaches which I didn’t have before): I assumed it was because of my Crohn’s but because of the different symptoms a part of me thought it had to do with my liver. And after some tests it showed that I have inflammations in my liver. All my liver enzymes are too high (mostly ALAT, Gamma-GT and alkaline phosphatase) except bilirubine. Also my platelet (thrombocytes) are too high. They assume it’s because of PSC but because it’s not ‘the typical example/picture’ of psc they don’t know for sure. Anyway… because of this I have some questions. I hope some of you can help me out!

  1. First of all (I’ve already discussed this with my doctor who thinks it’s psc and not something else) can it be something else entirely? What?
  2. The inflammation is inside the liver so they say the problem right now is small-duct psc but they diagnosed me with ‘normal’ psc. Is it possible that you can have both but only the small-duct psc is active? (at this moment). Also my MRCP mainly showed narrowing of the right hepatic duct. This is an intrahepatic duct (I believe); so wouldn’t that mean that I only have small-duct psc? I can’t really find much on the Internet about it! Only that people with Crohn’s disease are more likely to have small-duct psc.
  3. Right now the doctors say they can’t really do anything about the inflammations. I’m curious, what do you guys think from your own experiences, is there something that can help? Did any of you also had inflammations in the liver?
  4. I’ve heard two things that could be quite promising for me and other people with Crohn’s disease who had the unfortunate double diagnose. I’ve read somewhere that Vedoluzimab might be helpful for psc. Have they ever really followed up on this/do they have results? And I also read somewhere that people with Crohn’s disease have a better outcome and progression of psc. Is this true? I’m really curious about personal stories from people with Crohn’s!
    And if so, I’m glad for us who have Crohn’s but I’m also very sorry that people with colitis don’t seem to have that “luck”. I don’t really want to call it luck because I think none of us are lucky. And I’m sorry for everyone who has to deal with this very strange disease… with the pain, the uncertainty and everything around it. I hope all of you still have things in your life that make you smile and want to enjoy life as much we are able to!

Sorry for my long message! Right now it plays a rather big role in my daily life, resulting in much text haha.

Anyway, best of luck for all and I hope some people can answer (some) of my questions.

2

Hello Sophie and welcome to this PSC Community. I’m glad you gave us an introduction to yourself and what you’ve been facing of late. In reading over your questions one immediate question comes to mind that you might want to ask your doctors about. The only way to say absolute 100% that you have PSC is by having an ERCP procedure. You did not mention that in your observations. An MRCP is a very important diagnostic tool and provides a lot of useful information, but there’s nothing like actually getting inside those ducts and looking around. I’m glad your bilirubin is not high right now, but if you truly have PSC I think your bilirubin would show out of normal range prior to your first ERCP at least. --Are you under the care of a Hepatologist or just a GI doctor? If just a GI, I would encourage you to see a hepatologist who will surely want to order further tests such as the ERCP to verify diagnosis of PSC.

We have a number of folks in this group that either have Crohn’s and PSC and/or UC and PSC so you are not alone. Please continue to post and participate in the forums and feel free to ask anything that comes to mind. We are here for you to help in any way we can. Take care.

Mark
PSC 2011 / Liver Transplant 2015

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3

Hi mark,

Thank you for your quick reply!
I didn’t know about the ERCP, I thought they only did that if the images on the MRCP weren’t clear enough.
And I agree about the bilirubine which personally made me question the diagnose as well! But I’ve been under the assumption that I have PSC since December 2014! Ugh it’s a shame when they don’t know for sure but they still just diagnose you.
I’m indeed under the care of a GI but I’ve also seen a liver doctor (I think it’s the same as a hepatologist?) But he also continued to say it was PSC and didn’t do any more tests!

I think I’m going to make a new appointment and ask them to diagnose further…
Wouldn’t it be the best to do a liver biopsy? What do you think? They can rule out so many things at once. I just want to have some clarity!

Thank you for your help. And hopefully I’ll know more soon (or probably not very soon but yeah).

Take care and have a nice day,
Sophie

4

Sophie,
Hello again. In the early stages of PSC there is no damage to the liver to speak of. It is only after a time when your bile ducts become beaded which slows down the flow of bile, then the bile has no where to go. At that point slowly but surely cells in the liver begin to die. Over time that turns into cirrhosis and at some point you will need a liver transplant. If you are early stage diagnosed I don’t think a biopsy would serve in your best interest. You will know your liver is becoming damaged when you start having bile duct complications and they have to go in more often for ERCP’s to clean out the ducts. Once they do an ERCP the first time and for sure know you have PSC I would hope they would put you on Ursodiol which helps thin the bile so it flows better through the beaded ducts. Just take one step at a time. It’s not the end of the world and “if” and “when” the day comes you need a liver transplant, we hope and pray that a perfect match will come available for you as it did for me. Keep in touch and do follow up with your doctors. Please DO NOT let your regular GI doctor do the ERCP, but demand that it be done at a major hospital that does liver transplants. They have advanced endoscopist physicians who do this day in and day out. This is a very invasive procedure and in the wrong hands you could have some serious complications. In the right hands, usually everything goes smoothly. I had 5 of them over the 4 years I was diagnosed with PSC. Kind Regards.

Mark

5

Thank you so much for all your help! I really appreciate that you take the time to understand and explain things to me.
As I understand from what you are saying, it’s not likely that the inflammations in my liver are because of PSC. So I’m extremely curious what else it could be and why the doctors basically instantly label it with my psc.
I will follow your advice about the ERCP and a advanced endoscopist physicians! When it comes to my Crohn’s disease my GI is one of the best and they have provided me with great care, but I feel that PSC is too unknown for them which makes it harder!

I do have one question (if I indeed even have psc which I’m not even sure about right now) about the small-duct psc, which I asked before. About the right hepatic duct; do you or someone else know the answer to that question?

I’m very sorry to have read that your PSC had progressed so fast but I hope you are doing better and have a much better quality of life since your transplant!

Greetings,
Sophie

6

Sophie,
To my understanding small-duct psc are the bile ducts up in your actual liver whereas the larger ducts are outside the liver going down to your common bile duct where your pancreas ties in. There are two branches of bile ducts coming off your liver. The left and Right hepatic ducts. The right side is the larger of the two if I remember correctly. Bottom line in my thinking, you either have PSC or don’t have PSC. The small duct PSC seems to progress more slowly than the other but the outcome eventually is the need of a liver transplant, but there are folks in this and other forums who have had PSC for a very long time and still haven’t needed a transplant so it’s a matter that is very hard to predict. These are good questions but a hepatologist will be the one to get guidance on. Just so you can have something to compare your MRCP with, here are 4 of mine, one year apart. The first one at initial diagnosis and the last one 2 months before my transplant. This will at least give you an idea of what I went through. Just remember everyone’s different. Don’t let any of this panic you, you just have to live one day at a time.

I went back to one of my earliest MRCP’s and here’s what they said about me at that time back in 2011.
"Mild intra and extrahepatic biliary duct dilatation with mild irregularity of the intrahepatic bile ducts, consistent with patient history of primary sclerosing cholangitis. No dominant stricture.
2. No evidence of cholangiocarcinoma.
3. Mild splenomegaly, unchanged.

Now here’s one a year later…
MR Abdomen:
The liver is normal size. There is no significant hepatic steatosis.
There are no visualized hepatic lesions. The portal vein and
hepatic veins are patent.

The common bile duct is normal size. There is a mild beaded
appearance particularly of the central left intrahepatic ducts. There
is minimal intrahepatic biliary ectasia centrally, similar to the
prior studies. There is a focal apparent narrowing at the proximal
left intrahepatic duct best seen on series 1900, image 1. This is
very similar to the prior study and likely to the older studies
however this is less well visualized on the outside older studies.

There is no ascites. The spleen is enlarged measuring 15 cm.

The gallbladder may be slightly thick walled however it is
nondistended. The common bile duct is normal size. There is a mild
beaded appearance particularly of the central left intrahepatic
ducts. There is minimal intrahepatic biliary ectasia centrally,
similar to the prior studies. There is a focal apparent narrowing at
the proximal left intrahepatic duct best seen on series 1900, image

  1. This is very similar to the prior study and likely to the older
    studies however this is less well visualized on the outside older
    studies.

The pancreas parenchyma is normal signal intensity, and there is no
pancreatic duct dilatation.

The adrenal glands are normal. There is a single left kidney which
appears unremarkable.

There is no definite lymphadenopathy.
IMPRESSION:

  1. There is minimal intrahepatic biliary duct prominence centrally
    with a slight beaded appearance, consistent with a history of primary
    sclerosing cholangitis. There is apparent narrowing at the proximal
    most left intrahepatic duct which is similar to several prior
    studies. This may represent pulsation artifact from the hepatic
    artery. No focal mass is seen. Continued followup is recommended.
  2. Splenomegaly measuring 15 cm. Previously measured 14.7 cm.

Now another MRCP a year after the one above…
Findings:

There is no pleural effusion.

There is smooth hepatic contour. There is no signal dropout on post-phase
images to suggest hepatic steatosis. There is persistent splenomegaly
worsened from prior, now the spleen measures up to 16 cm from prior of 14.5
cm. There is no definite focal hepatic lesion. There is heterogeneous
perfusion on late arterial and portal venous phase images of the anterior
right and medial left hepatic lobe similar to prior exam. There is
otherwise normal perfusion of the hepatic parenchyma. On delayed post
Eovist enhanced images there is biliary excretion for example seen in a
single right hepatic ductal branch however there is no excretion within
gallbladder or other intrahepatic, extrahepatic ducts.

There is an accessory left hepatic artery arising from the left gastric
artery.

There is a persistent beaded appearance of intrahepatic biliary ducts with
multifocal mild intrahepatic biliary ductal dilatation, more pronounced
from prior examination. There are again-seen areas of increased T1 signal
again seen projecting in the region of the right hepatic lobe may represent
a small amount of sludge. There is no filling defect or central obstructing
mass. Common biliary duct is nondilated measuring up to 0.3 cm. Gallbladder
is decompressed and there is persistent pericholecystic fluid.

Pancreas is of normal signal intensity no focal pancreatic lesions or
pancreatic ductal dilatation.

The right adrenal gland is identified appears unremarkable. There is a
single left kidney visualized which demonstrates no hydronephrosis or other
abnormality.

There is a small para-umbilical hernia measuring 2.3 cm x 3.4 cm now with a
small amount of fluid.

No free pelvic fluid or bowel dilatation.

Impression:

  1. Worsened intrahepatic biliary ductal dilatation with beaded appearance
    likely representing disease progression. No extrahepatic ductal dilatation.
  2. No evidence for cirrhosis or focal lesion.
  3. Decompressed gallbladder with pericholecystic fluid, unchanged from
    multiple priors. Please correlate clinically for cholecystitis.
  4. New fluid within known para-umbilical hernia, correlate for symptoms.
  5. Persistent perfusional abnormalities of the liver as above likely from
    chronic hepatic inflammation / edema.

Now this is the last one prior to my transplant just 2 months before.
Findings:

The visualized portions of the lung bases are unremarkable. The liver is
nodular in contour. Irregular intrahepatic biliary ductal dilatation is
again seen, compatible with history of PSC. This appears increased compared
to prior study from October 20, 2014. The CBD is normal in size and
measures up to 0.4 cm. There is a likely short-segment stricture of the
intrahepatic common bile duct at the level of the bifurcation (series 10
image 17). There is no mass seen in this region. The main portal vein
remains enlarged and is patent. The hepatic veins are patent. No focal
hepatic lesions are identified. Areas of increased signal intensity on
arterial phase images within the right hepatic lobe are favored to be
perfusional in nature. Gallbladder wall thickening is again noted. Replaced
left hepatic artery arising from the left gastric artery.

Splenomegaly is noted, measuring up to 16 cm. Multiple upper abdominal
varices are again seen. There is recanalization of the umbilical vein.

The left kidney is normal in appearance. No right kidney is seen. The
bilateral adrenal glands are unremarkable. The pancreas is normal. No
lymphadenopathy. Umbilical hernia is again seen, which contains fat and a
small amount of fluid. The visualized bowel is otherwise normal.

There are no aggressive osseous lesions.

Impression:
Findings compatible with primary sclerosing cholangitis, with short-segment
stricture of the common bile duct at the level of the confluence and
increased intrahepatic biliary ductal dilatation compared to prior study.
There is no definite mass at the level of the stricture, though a small
mass at this location cannot be entirely excluded. Correlation with ERCP is
recommended. No evidence of portal venous occlusion. Redemonstration of
findings of portal hypertension.

Kind Regards,
Mark

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7

15 years ago we used to diagnose PSC with invasive tests. Today, MRCP imaging has improved to the point where it is the gold standard test for diagnosis. ERCPs are typically reserved for when therapeutic intervention is necessary like a blockage that can be opened up. Diagnostic biopsies aren’t usually necessary when PSC is clearly visible on MRCP, but can be useful if AIH overlap or small duct PSC are suspected.

If the ducts show a beaded appearance on imaging then this points to classic PSC (the report will say something along the lines of “…is consistent with PSC”). Classic PSC can affect the ducts inside (intrahepatic) and outside (extrahepatic) the liver. Notably, classic PSC can be seen via imaging. Small duct PSC only affects the smallest ducts inside the liver - those that are too small to see on imaging (think of the bile system like a tree - small duct PSC only touches the smallest twigs). If symptoms and liver function tests all point towards PSC but nothing is showing up on imaging, they can perform a liver biopsy to attempt to find small duct PSC.

The 50 million dollar question is how to get rid of the inflammation. We don’t have any definite answers, but there’s a lot of ongoing research attempting different approaches. Drugs that are being researched and that can be taken off-label for PSC include urso, oral vancomycin, and vedoluzimab. There are a number of other drugs in the pipeline but they can’t yet be taken outside of a trial. I personally have had success with oral vancomycin. As a side benefit, many PSC/UC and PSC/Crohn’s patients are able to eliminate their other IBD meds when on this antibiotic treatment.

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8

Thank you Mark and jtb,

I’m so delighted and grateful how kind and helpful all of you are, it’s truly amazing! Especially because people around me (except partly my mother) don’t really understand or know about the disease.

Mark, thank you for showing such private reports and for making me understand it all a bit better! Your reports a very well detailed and informative but I can imagine that it was pretty tough reading those reports. I did find some similarities with mine reports. It’s all in Dutch and I don’t think I’m able to translate it without it being a total mess. But seeing your reports helped me to understand mine a bit better as well! (As far as I can understand both reports). I think it’s true what someone had said on this site; you have to be your own expert on this disease.

Jtb, thank you so much for your clear explanations!
My first report said: image could very well be fitted with psc.
Second report: image fitting/compatible with psc.
I’ve translated it but this was basically what they said (after a detailed information of their findings from my mri liver and mrcp).
And the medication seems promising, it’s great news that they have trials. I am on Vedoluzimab so just hope it will suppress my Crohn’s disease and psc for a long time.

Now I just have to figure out (with a good hepatologist) why I have inflammations in my liver!

The hardest part about psc is knowing it will progress but not knowing when and what will happen. That scares me but I already feel a bit better after your explanations/information! It’s such a relief to talk to people who, from personal experience, understand this disease. Because so far there hasn’t been anyone who understands or can help me.
You help me to make the situation a bit less scary! So again, thank you very much!

I’ll let you guys know if I know something more concrete!

Kind regards,
Sophie

9

Good morning Sophie,

Are you aware of the Dutch Facebook group on PSC and AIH Overlap?
https://www.facebook.com/groups/PSCenAIHoverlapNederlandstalig/

Maybe you want to consider joining.

Kind regards
Marleen

10

Hi Sparkles,

Thank you for your message! No I wasn’t aware of any Dutch psc/aih group on Facebook! I don’t have Facebook but it might be something to check out!
Do you know if there is also a Dutch psc group?

Anyway thanks for your idea!
And may I ask, are you a Dutch PSC patient as well?

Have a nice evening!

Kind regards,
Sophie

11

There’s agroup called PSC en AIH (overlap) Nederlandstalig. It is a closed group, but you can ask to be a member.

Yes, we occassionaly meet, keep each other posted etc… We are trying to get more attention for PSC in NL and beyond

Have a good evening
Marleen

12

Hi Sofie,

I just wanted to chime in and say that I agree with JTB that MRCP is now preferred method for diagnosis. ERCPs are useful tools, but come with potential risks and should be saved for times when the docs actually need to get inside the ducts.

My MRCPs only show issues in the intrahepatic ducts. I found this info which explains that 10 percent of PSCers have intrahepatic only, 5 percent have extrahepatic only and the rest have both intrahepatic and extrahepatic. http://www.hopkinsmedicine.org/gastroenterology_hepatology/_pdfs/liver/primary_sclerosing_cholangitis.pdf

Take care!

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13

Hi crewmom,

Thank you for your reply! :slight_smile:
And I believe that my MRCP’s also only show issues in the intrahepatic ducts, mostly in my right hepatic duct.
How are you doing? Have you ever needed an ERCP? Or do they only use those for extra hepatic ducts like the common bile duct?
I think it’s ‘better’ if we only have troubles with the intrahepatic ducts! For example, we have less chance of getting cholangitis attacks. This is an assumption because I don’t know if there is a big difference tbh! Maybe I’m completely off but from what I’ve heard this is the assumption I made.

Anyway, thank you for your information and I hope you are doing alright!

Have a good night,
Sophie

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14

Hello, sorry you have this disease that basically has no treatment other than deal with the symptoms when they occur. It is very frustrating to be told no treatment and no cure. I started to have symptoms in 2015, itching, loss of appetite, pain in upper right side and a tight feeling in upper abdomen, which I found out that is the fibrosis of the liver. I have stage 3 fibrosis. My alp was over 700 and the ast and alt were about 125. I had erythema nodosum, which is swelling and lumps on my ankles and legs. At that time I was given Colchicine and my alp started going down. Since I have been taking it my numbers went down to 188 on the alp and around 25 on the ast and alt which is a very significant drop. The swelling went down on my legs and my appetite returned. I was given ursodiol later last year and am on both drugs now and am holding steady on my numbers. I really don’t know if it is slowing the progression of the disease or not, but I have a better quality of life since taking it. Will be getting a MRI next month to see how the bile duct are doing so that might show if the drug is slowing things down. Best of luck to you and hope you and everyone dealing with this. The Colchicine has really been a wonderful treatment for me and hopefully others will try it to see if it helps them. I know it is not usually given for PSC and it was even in a clinical trial but some drugs may help some and not others.

15

You are all so kind! It’s truly amazing and it helps to talk to people who understand what you are going through! Even though all our stories are personal and different, I think all of us deal, or have dealt, with the uncertainty and the physical and emotional pain of being diagnosed with this rare disease.

Thank you for your personal story petals and your kind words. I’m sorry that you had such a bad start with this disease, but I’m glad you are feeling so much better now with the medicine! Whatever the reason and whatever will happen it is great that as of this moment you have a better quality of life.
I’m sorry you have erythema nodosum but it’s also quite interesting for me; because I have it too! I know how utterly painful and annoying the swelling and lumps can be! I only have the lumps all over my ankles and lower leg whenever I have a very bad flare up because of Crohn’s disease. It does all seem to connect somehow… and apparently you had it when your PSC was active!
I didn’t get Colchicine (never heard of it before) because whenever they treat my inflammations and stenoses in my small intestine/colon with prednisone, TNF-blockers and surgeries the swelling/lumps went down as well and disappeared.

I do use Vedoluzimab because of my Crohn’s and they say it might be helpful for psc as well, so I hope it does! But as you said it totally depends on the person. The disease affects everyone differently and that is very frustrating!

Good luck with your mri!! Let us know the outcome!
I truly hope the images on the mri will show that the disease somehow stayed stable and that you will have a good quality of life for as long as possible!

Take care and best of luck with everything :slight_smile:

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16

Hi Sofie,

I’m pretty much a “lurker” here. My son (now 22) was initially diagnosed in August 2016, based on elevated liver enzymes, followed up by ultrasound and MRCP. We were referred to a NYC gastro for a possible ERCP, but he chose to do a colonoscopy instead in December. At that time he formally diagnosed Crohns and PSC. Since the beginning my son has not had any symptoms for either Crohns or PSC. He feels great, is in college and seemingly dealing with everything. However, when I discussed the findings with the gastro in December about possible medications, he said that there is nothing to do for the Crohns as there are no symptoms, and that there are no treatments for PSC.
Although that may be true, I could not just sit around waiting for something to happen. I got in touch with a heptologist/liver transplant doctor at Weill Cornell in NYC. At the visit, he pretty much confirmed everything based on all the records. I brought up vancomycin, and he was pretty neutral on it. But, he was more than willing to prescribe it if I wanted. But, he wanted me to look into other trials, including one that his group will be involved in. If my son started vanco he may be ruled out for other trials.
Turns out, the trial he was thinking of is not ready to start.
At this point, because my son’s enzymes are higher than normally seen with PSC (his bilerubin is normal), he wants to do a liver biopsy to rule out AIH. We are trying to see when that can be scheduled (March or May, based on my son’s school schedule).
If my boy does not have AIH, I plan on getting him on vanco ASAP.

Best of luck to you.

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17

Hi jace,

Thank you for your reply and your own personal story! I’m so sorry for your son and his double diagnose! I know how hard all of it is for my mother so I can understand how hard it must have been for you to hear about your son.
However, it is great news that he doesn’t have any symptoms and is doing so good! Why did he get tested in the first place if he didn’t have any symptoms?
I hope he will stay healthy and asymptomatic for a very long time. And hopefully they can give your son a medicine that can make all of this stable for even longer!

Best of luck to you and your family :slight_smile:

Kind regards,
Sophie

18

Hope you are feeling good yourself. Every summer, before returning to college, my son get a complete physical with blood work. This year, his liver enzymes were high, and that started us on the road…

19

Thank you Jace :slight_smile: At the moment I do have some symptoms (the most annoying one at the moment is the constant fatigue) but thankfully it’s manageable!
My PSC story is sort of the same: I also got diagnosed because of high liver enzymes.

I wish you the best of luck with everything and hope your son will stay healthy for a very long time!

Kind regards,
Sophie

20

Thank you, will let you know the results of the MRI. My daughter has been diagnosed with PSC also which has been so much to deal with. We are hoping the diagnosis is wrong, she has inflammatory arthritis too, and is in a lot of pain from that. Wishing you all well, good luck with your treatments.