Crossover syndrome

Hi I’m seventeen years old and I was diagnosed with a crossover syndrome between autoimmune hepatitas and scelerosing cholangiatas . (ASC)

Is there anyone here that has the same thing as me?

Hello Jaiken, and welcome.

I'm a geezer compared to you, nearing 50. I'm not very familiar with ASC, but last year I was diagnosed with UC, PSC, autoimmune hepatitis, and cirrhosis. My doctor has put me on an immunosuppressive drug call Imuran (azathioprine) to help control my UC and autoimmune hepatitis. He put me on Urso to help with the PSC. It took several months to start working (in the meantime I was on Prednisone), but we did see my Liver Panel numbers come down to a closer-to-normal range. I have since been weaned off the Prednisone (grateful for that!) and my numbers still look pretty good. I go back in June/July for ultrasounds and more bloodwork to see how things are shaping up.

Before being put on the immunosuppressive, my doctor had me get vaccinated for everything under the sun. He also warned me that I was likely to catch many of the common maladies going around as my immune system may be too weak to fight them off. I run a small business and do a lot of meeting people. At 17-years old, I imagine you are still in school (but who knows, you could be brilliant and running a Fortune500 company right now). Being around large numbers of people increases the chance of catching colds/flu/whatnot. There is only so much we can do, but I must say that I have made hand-washing a big part of my day. I've been lucky and not come down with many of the bugs going around. On the downside, I've become pretty obsessed with hand soaps. Quick Visual: Imagine a burly working-class southern American sniffing hand soaps in the pharmacy isle.

I hope all is well with you and that your physicians are setting you on a path to wellness.

I'm curious to know your treatment options with ASC and more about ASC altogether.

Have a great day.

Hi Jaiken,

I am sorry to hear about your diagnosis. I don't have overlapping autoimmune hep, but I know it's not terribly uncommon and seems to be more common in pediatrics. I wanted to let you know in case you aren't aware that there is a young adult support group on Facebook.

https://www.facebook.com/groups/234653753345838/

It's a closed group. There are also some other, larger support groups, too. And PSC Partners Seeking a Cure is a great place for information. Good luck to you!

Crewmom

I'm sorry to hear of everyone's disease. Our daughter was recently diagnosed with AIH-PSC overlap and is taking prednisone (trying to ween off), URSO, and mercaptopurine (Azothioprine derivative I believe).

  • Does anyone know of anyone using Vanco for AIH-PSC overlap?

Thank you.

Hi Ike,

I can’t answer your question but i do know that dr kenneth cox of stanford university might be able to. The 2 times i called his office he called me back and answered my questions. (His name and # comes right up on google)

The best to you and your daughter
Jam

My son was 12 when he was diagnosed with AIH and PSC ( overlap ). He was stage 3/4, the PSC was in the beginning stage. He is 15 now. His specialist started him on 60 mg of prednisone, followed by 100 mg of Mercaptopurine( MP6 ). His liver enzyme numbers came down to twice normal after 9 months. He was weaned off prednisone gradually. His numbers are still twice normal after 2 years. He will be getting a liver biopsy in August to see if his liver is better of worse and then to either stay the course of start something else.

Concernedmom,
When was the last time your son had an ERCP and what comments did the doctor make afterwards to give you an indication of the condition of his bile ducts. Is he on Urso and how is his itching these days. Hoping he continues to do well and those numbers stay within reason. As you know, with PSC the liver enzymes will always be high.

Mark

He had an MRCP at the time of his biopsy that led to his diagnosis at the end of 2014. I asked our family doctor to run some blood work because I thought he looked slightly yellow. He had no symptoms other then not gaining weight. We were sent straight to a pediatric gastroenterologist and hepatologist.
He still has no symptoms. His symptoms have been from the drugs. He has had constipation at times.
The liver biopsy results read, " focal periductal rimming by fibrous tissue ( onion skinning), focal mild interlobular bile duct damage, and focal ductular reaction." He had a normal colonoscopy and endoscopy.
His liver enzyme numbers went from ALT in the 800 range down to 71. My confusion stems from him having no symptoms, yet his blood work does not show him getting to the point that he can get off of Mercaptopurine. He is not on any other drugs. I am starting to think we were mislead to believe he would go into remission for 2 years and be done. I wonder if PSC can progress without symptoms.

One clarification I’m trying to get at and perhaps I just am not being clear, but one important question I keep coming back to is has he had an ERCP procedure? You mentioned a normal colonoscopy and endoscopy but with the endoscopy are you referring to just a normal EGD test that the GI does on you from time to time? That’s not what I’m referring to. This is an advanced endoscopic procedure that goes down into the stomach, and then back out into the common bile duct and then up into the right and left hepatic ducts leading up to the liver. With PSC this is what get’s strictured (blockages) that leads to elevated LFT’s such as high bilirubin and alk-phosphatase. If he hasn’t had one of these done yet that needs to be done, and is really the only sure way to confirm with accuracy true PSC diagnosis. Now he may have had that done but that’s what I’m trying to establish before saying more. With PSC that procedure will have to be done from time to time to give some temporary relief and allow the bile to flow. Over time the procedures will yield lesser results as the damage to the liver begins in earnest. With PSC you start out with a perfectly healthy liver in most cases but it’s the bile ducts that get the disease which when they disease it restricts the flow of bile and then over time that is what destroys the liver. If he has not had one of these done and a decision is made to do that, make sure you ask the specialist at the liver transplant center that does these every day to do the procedure. This is not a procedure to just allow your local hometown GI doctor to do as it is very invasive and can cause serious complications in the hands of someone that is not an expert in it. I know that’s a lot but I hope it helps some. Looking forward to helping in any way I can.

Mark
PSC 2011 / Liver Transplant 2015

No he has not had an ERCP. He had an ultrasound, liver biopsy, MRCP, colonoscopy and endoscopy. The AIH was the biggest concern as he was already stage 3-4 diagnosed from 2 needle biopsy’s. The information on the bile ducts that I quoted in my last post is all I know about the PSC. He is under the care of a pediatric gastroenterologist/ hepatologist formerly at Texas Childrens Hospital. My son’s results were evaluated by two different pathologists from separate hospitals. I have read that having this combination of overlap is rare and there is not a lot of research. He has no symptoms of the PSC so I imagine that is why an ERCP has not been scheduled. Am I correct that the PSC can take a lifetime to cause problems? ( we know it complicates getting and keeping him into remission for the AIH). I appreciate your insight, I am finally able to talk to someone who has heard of this.

Can you tell us an update? My son is now 15 with the same diagnosis.

It is true especially in most people who get PSC when they are young that the rate of progression tends to be slower than in adult onset. Hopefully he will have many symptom free years to come. Keeping up with his labs every 6 months or so, not sure at that age if it’s less frequent will give you a good idea of progression. One thing I did over the years is to track all my labs in a spreadsheet so I could see trends in my PSC progression. At some point if his bilirubin really starts to climb up into the 2-4.0 range and he has a lot of itching, lower right back pain, etc. then it’s probably time to be talking an ERCP. They will want to put him on URSO at some point which helps to thin the bile. It brings more quality of life than anything else as there is no current cure for the disease. Not all doctors believe it works but there are many patients who will tell you it greatly improved their quality of life which is most important. MRCP’s as he gets older will probably be done once a year to track his progression. Encourage him to stay as active as he can and just live life, plan for the future like college and marriage hopefully at some point. It’s not the end of the world. When you feel bad, you just slow down, rest, get intervention and then just continue on. There is hope and there is help and we can only pray that one day there will be a cure.
We are here for you to help as we can. Take care.

Mark

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Thank you for that information. I think a spreadsheet is a great idea. He has labs done every 3 months and the results are all over the place. His doctor has a spreadsheet and graph, I will ask if he will copy it for me. I will pray for a cure.

I’d also be glad to send you a copy of one of mine and you can just delete my entries for your own. It may give you an indication of how wild the numbers can go up and down. I should have your email in your profile so I could privately send that if you desire. Just let me know.

Mark

We’ve been on this journey with our daughter now for about 1.5 years, (PSC-AIH-UC). Her liver numbers are normal for the past 8 months. Her UC acts up from time to time. She is currently on URSO, Azothioprine, Mesalamine, vitamin, Budesonide (less side affects than Prednisone). We pray for years of good liver numbers. We are learning to follow Mark’s advice of living life and dealing with issues when they arise. We use a spreadsheet to organize the lab data and to help track changes in medication. Our daughter’s attitude remains positive and she does well taking her medicine.
We wish everyone well and thank everyone for their support.

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Yes please do. I marked my time by thinking after 2 years of remission from AIH this would be over. Needless to say it took 9 months to reach that status and it has been unstable for almost 2 years since that month. Finding out more about the PSC I realize I must adjust my thinking. I am going to track this from now on.
He is very active, very happy. We just go with the flow when he falls asleep in between his activities.
Thank you.