My son’s (5 yr old) liver biopsy was just read by a second pathologist who indicated they are “Very confident” this is case of overlap syndrome, Autoimmune Sclerosing Cholangitis (ASC). Stage 3 fibrosis (more advanced scar issue), No cirrhosis.
He was put on Ursodiol/ Prednisone about two weeks ago, and we got him on vanco (pills-generic) about 9 days ago. We are not breaking the pills apart at the present time, but I did read some of the posts on this issue and will consider it in the future (or potentially switching to the liquid form).
Overall, his liver numbers are down but still elevated. ALP has come down the most (about 68% since beginning treatment) and is now borderline normal. AST is down 59%, but still 2x normal. ALT is down 18%, but still 4-5x normal. GGT is down 39% but still 13x normal. PT/INR has gone from above normal to normal. Bilirubin has never been elevated.
Other than ALP, there has not been any material improvement since beginning vanco, but it has been just 9 days.
On the positive note, my son has a ton more energy, gaining weight and appetite (likely because of the steroids), more outgoing, etc. His stool is now normal color (from pale), no more diarrhea. We are likely going to reduce his prednisone soon and add azathioprine.
The first read of the biopsy by a different pathologist and GI indicated his condition was “more PSC” than autoimmune hepatitis. Now, it appears this is a clear case of overlap syndrome, along with IBD (UC).
The liver specialist who provided the above diagnosis is not opposed to vanco (we have another liver specialist who prescribed it) but is not sure if it will work with overlap syndrome.
Questions:
Is overlap syndrome a 'better diagnosis" than pure PSC-IBD?
Does oral vanco work in your experience with overlap syndrome (particularly children with IBD/UC)?
When should we expect to see more dramatic reductions in the liver enzymes above if vanco is working? Any way to know (or guess) what medications are impacting the above enzymes/GGT, etc.?
Any other advice or comments to the above is greatly appreciated.
I wish I could comment with more insight, but I am very new to this forum. I was only recently diagnosed with PSC or overlap syndrome (there still seem to be some gray areas for my care team, which has been a theme since my UC/autoimmune hepatitis diagnosis in 2010). Being new to this forum, I only recently learned about vanco as a possible therapy, and will look forward to hearing how your son responds over time. The main reason I wanted to respond, however, is to express how deeply your post touched me on a maternal level. I cannot imagine what it would feel like to be in your shoes, fighting to find answers and relief for your precious child. I see how hard you’re fighting for him, and will be praying for your whole family as you carry him through this. Also, thank you sharing his clinical picture in such great detail, so that others (like me) might be able to learn how to better manage this disease in the future. Big hugs to your son and family.
Also, for what it’s worth, you’ve raised some great questions for me to ask my hepatologist at my next appointment. If she shares any helpful insight, I’ll be sure to follow up.
For what it’s worth, the first 3 days (partial fill) of vanco, was Lupin (manufacturer)–pills. The next prescription was through Alvogen (manufacturer), which I am learning has not worked as well for some others. We are working on switching to ANI pills. He has been a champ taking the pills and medication.
I haven’t kept up with the latest on PSC/AIH overlap. There was limited data years ago and the thought at the time was that overlap patients had better outcomes. The speculation is that this was due to the fact that overlap patients, unlike pure PSC patients, tended to respond to steroid treatment.
Anecdotally, OV treatment appears to work with PSC/AIH overlap patients. The 2008 Davies long-term treatment study included 12 patients with autoimmune markers (4 ANA, 4 ASMA, 4 p-ANCA). I’m not sure if there were any official overlap diagnoses.
Notably, all 12 patients subsequently tested negative for autoimmune markers while on OV treatment.
GGT is slower to respond compared to ALP. In my case it took something like 45-60 days for GGT to normalize after ALP normalization. My team would give things 2-3 months to see what happens before making any major adjustments.
Some people do great on the pill generics while others benefit by switching brands. In my case Alvogen brought my ALP and ALT from the 600s to the 200s, but with no further improvements over many months. Switching to an IV compound brought my ALP and ALT down to normal almost immediately and these stayed normal after switching to pill form Vancocin (now sold by Ani). The gel caps used by generic brands dissolve more slowly at particular pH (in this case, a subset of patients) and the speculation is that this may be the problem.
Patients with overlap syndrome have shown to respond to vancomycin but the response may take a few months. Data is not plenty to does exist. Having AIH obviously complicates treatment.
As an update, we switched to ANI last week. We were told we need to choose the liver specialist who we would prefer to work with so we went with the one that believes in and prescribed Vanco. We are slowing weaning off of Prednisone and are not going to go with azathioprine. We added a medication for UC. I will post updates on labs going forward and progress.
As an update, since beginning medication July 21 (and Vanco on July 25), my son’s ALP is down 72% (now normal range), AST down 65% (still above normal but getting closer to upper limit), ALT is down 35% (but still elevated) and GGT is down 50% (still elevated). Every other test pretty much normal or near normal. We switched to Vanco manufactured by ANI 5 days ago. All of these markers had substantial reductions over the last week. Not clear if this is due to Vanco or Ursodiol, but very happy with his labs today.
Hi there - I just saw this post but see it has been a month or so since posted. I am also the parent of a young psc patient. I would love to connect with you and talk more. Are you also on the PSC Partners Seeking a Cure FB group? Have you found Www.pscpartners.org? There is a lot of very important and up to date info there and many others in your shoes as well. If you would like to connect please PM me - let’s find a way to speak. Hang in there.