PSC or Not, Try to understand what is it

Life with Primary Sclerosing Cholangitis
1

Hi to everybody,

I am Ozgun from Turkey. It is great to have such a communication tool for everybody. Let me try to explain my case.

While I was getting medical control for military exams on 2006, It was seen that my ALT and AST was little higher than the upper limit which was considered OK at that time. Anyhow, I didn’t continue my application to army, I choosed engineering education instead I was 18 at that time). But we tried to understand why was my liver enzymes test was higher. I remember, they first checed hepatit markers which was found ok. The only thing they found abnormal was ALT AST, total and direct bilirubin (lower than 2 times higher approx.) and ALFA-1 antitripsin was low. They first though it is Wilson, they checked copper in 24 hours Urine test which found I remember 2 times higher than it shall be which was not strong enough to go further for them I think. Meanwhile, you can sure they checked everything since It is a fully fledged university research hospital. I has liver biopsy on 2006 which gave us only result with ‘fatty liver’.

Since this researches became a back and forth goings to hospital, I bored to continue since I didn’t have a sufficiant diagnosis. They lastly said It can be NASH. But I followed it 10 years regular base. In that time, We didn’t record a meaningful change on liver enzymes. For a while, I used Ursofalk which helped to decrease my enzymes to normal. But afterwards, doctors said me to give up since UDCA doesn’t create a permenant solution.

In 2015 October, I experienced first time an obstructive jaundice, all ALT, AST, ALP, GGT levels were about 250-400 and so on while bilirubin levels were about 4 If I am not wrong. Within a week, I became normal without any medical or surgical intervention. My doctor warned me to go emergency if I have a cholangitis atack ( fever and chills basicly) which I didn’t. After a week, enzymes were back to 2-3 times higher level. Afterwards. My doctor did me ERCP intending to put a stend on my bile duct by considering me as a PSC patient. I wasn’t of course aware of what PSC is at that time, but my doctor failed or decided not to put stend due to inflamation on bile ducts. But in ERCP report, It doesn’t state a supportive findings for ERCP. I had a MRCP after ERCP with below statements (I hope I translate well)

Focal dilatation spaces in peripherie intrahepatic bile ducts (for the petient who is being followed with PSC diagnosis, outcomes are supporting the diagnosis). Date: April, 2016.

The thing is here I wasn’t diagnosis with PSC at my ERCP but MRCP is kind of referring another diagnosis which I didn’t understand at that time.

Note: Meanwhile, First thing they did after ERCP inflammation detection was colonoscopy which found only a polip, no IBD findings such as UC or Crohn on February, 2017. Due to Colon CA history of my father, I repleced a colonoscopy after a year with normal findings.

He said me to use UDCA repeat the MRCP after 1 year. I repeated it with below result comment:
Minimal disorders in Intrahepatic bile ducts (focal dilatation spaces seen on last MRCP was nor observed in current one. While finding is non-specific, typic outcomes were not observed. Even though typic outcomes were not observed, If PSK is diagnosed to mentioned petient, outcomes can be considered to support the diagnosis)
Dated April, 2017

This last report is totally confusing me and my doctors. Meanwhile, my blood results became normal or sligthly higher than limit after UDCA. My doctor is confuced since he said PSC normally doesn’t show us better results, it is a progressive disease. They all say continue to use UDCA and repeat MRCP yearly.

This uncertinaty is really making me sad. It would be appreciated to see your comments since I am really obsessed with it, I feel that I am trying to create a supportive symptoms of PSC which is not good.

I hope I am OK with my English to explain my story.

Thanks in advance for your comments.

Greetings,

Ozgun

2

Hello Ozgun and thank you for posting your story. Everything you have described is very similar to my own experience with PSC. Often, when you are first diagnosed with the disease and the ducts are cleared by ERCP you can go symptom free for quite a while. I went a few years after my first ERCP before I needed another one. I too had MRCP’s annually with a visit with my hepatologist every 6 months including blood work. I was on URSO the entire time from diagnosis to transplant taking 1200 MG’s a day. Although it doesn’t reverse the disease it does provide a quality of life that makes it very valuable to our day by day living. Yes, PSC is a progressive disease but it progresses at different rates in each patient. Some patients will go many years 10+ with very few symptoms and then things will take a turn for the worse quite rapidly. I would encourage you to just live your life to its fullest while you are doing fairly well and when things change deal with it then. Just keep your appointments and take good care of yourself. There will come a time in your life that things will progress worse and you will need more intervention, more frequent ERCP’s etc. As long as your MELD score stays relatively low you will hopefully be in pretty good shape, although with PSC some patients MELD scores don’t rise with the level of their disease progression which makes it tricky in getting placed for transplant.

Let us know if you have any questions or concerns and this group will be glad to try and provide the answers you need.

Mark
PSC 2011 / Liver Transplant 2015

3

Hi Mark,

Thanks for your comments. Yes I actually have some questions by considering possible diagnosis of PSC for myself.

  • Since no IBD (UC or Crohn) seen on my colonoscopies, Shall I expect to see IBD in later stage of PSC or it is not directly related with PSC? Because, my doctor said me to repeat colonoscopy after 3 years.

  • I have a kind of hives seen as tiny red rush on my skin since February, 2017, appears as a bite of a fly and disappears after 15 min. Repats about each 2-3 days, very rare, normally seen on my right arm. I was wondering whether this is a sign of PSC progression, but my doctor said not related with liver, might be alergic. When I went to dermatology doctor, she repeated the same, she said this is typical kind of urticarea. I used aerius which removed the rushes. But when I gave up the drugs, it reappeared. I will again wo to dermatogogy doctor but I wonder whether can this be a symptom of PSC? Because I never experienced itching even though I had jaundice in 2015. Does UDCA have such a sideaffect? As far as I searched, it has very minor persentage (%1-3).

  • Last thing: I am still not fully convinced with the diagnosis because of difference in the reports. My doctor also chedked IGg4 for possible autoimmune pancreatit diagnosis which found ok as well. Are there any further blood test to identify it without waiting a yearly MRCP to ensure for the diagnosis? Because my ALP value is normal without using UDCA and I even heard one of a doctor which I went : ‘medicine didn’t identify each disease yet’ which is kind of annoying to me.

Thanks,

Ozgun

4

Ozgun,

As there is a strong connection between psc and ibd-usually UC, docs have to be careful. And there is an increased chance of colon cancer. Docs are smart to schedule colonoscopies.

But ibd is not something you should “expect” to see when you have psc. In my case, I had UC 11 years ago, 7 years before my diagnosis of psc. When I had my large intestine removed, goodby UC.

Itching is very common with psc. Some people have it worse than others. Itching in my legs and shoulders drove me crazy. There are some medications that can help.

There is not a blood test that can diagnose psc. With psc, and even trying to diagnose it, it will test your patience, sense of humor and your ability to be flexible enough to handle psc’s unpredictability. Because psc is considered to be rare, most docs have no experience with it. That is why it is a good idea to stick with hematologists/liver docs instead of general practitioners or even GI docs.

Jeff

5

Hi Jeff,

Thanks for your reply. I have read much about the strong relation between PSC and UC, but as I understand man cannot say both will appear in all cases. I am still not sure whether will I see UC in future because of possible PSC diagnosis. I think nobody can answer it at the moment.

I wonder whether you or someone else in the forum read or experienced red rushes on the skin disappears in a short while as a symtom of PSC. Even if my dermatologist said, this is not related with liver, I am curios to know that if this can be a pre-symptom of itching (those tiny rushes are not itching however yet).

Ozgun

6

Hello again,

I like to update you all guy for my story. I have prescribed used 1.5 year URSO (between November, 2016-April, 2018) after I had Obstructive Jaundice in November, 2015.

I had ERCP made right after Obstructive Jaundice, couldn’t complete the process due to inflammation on gallbladder. Afterwards, they started URSO for a while and had MRCP.

My MRCP in April, 2017 states as follows:
Minimal disorders in Intrahepatic bile ducts (focal dilatation spaces seen on last MRCP (2016) was not observed in current one. While finding is non-specific, typical outcomes were not observed. Even though typical outcomes were not observed, If PSC is diagnosed to mentioned patient, outcomes can be considered to support the diagnosis)

After this MRCP findings, my doctor was confused if this is PSC or not, however, he recommended me to continue using URSO and repeat MRCP after a year.

I did repeat MRCP on March, 2018 and reports says as follows:
Minimal dilatations on bile ducts with segment 4 level at peripherie localization.
Minimal disorders at Intrahepatic bile ducts (No major obstruction was observed for this case which is followed with PSC diagnose. No significant difference was observed when result is to be compared with the report of patient on April, 2017).

Based on this report, my doctors are once more confused and not sure about PSC diagnose since MRCP reports does not describe a progressive typical PSC signs such as obstruction. My last blood results:
AST 36, ALT 81, ALP: 72, GGT: 39, Total Bilirubin 2.13.

All other blood results are normal, including p ANCA, c ANCA, ASMA, AMA, LKM,APCA.

My doctors said me to drop URSO and come 3 months after. They are thinking It is maybe simple gilbert and that is all. I will ask them to repeat ERCP after 3 months.

I wonder if someone here had similar case or what are your comments on these. I am really tired to think about this over 12 years!

Thanks your usual support and interest.

Ozgun

7

Ozgun,
Hello and thank you for your post. The first thing that comes to my mind is this. Are you under the direct care of a transplant hepatologist at a major medical center that performs liver transplants? If the answer is no, then herein is your problem. What about the reports from your ERCP’s? What do they indicate? Were these performed by your local GI or an specialist in advanced endoscopy tied with a transplant center? I just wanted to emphasize how important it is to be seen ALWAYS with PSC by a hepatologist at a transplant center.

Mark

8

Hi Mark,

I have been tracked by one of University Research Hospital here which is the ones who are fully fledged hospital, and has both gastroenterology and hepatology policlinic with capable professors. They do transplant as well.

I don’t have in my hand now for ERCP reporting, but I can say the doctor’s wording “they couldn’t complete ERCP” because of inflammation on gallbladder ducts. It states pancreas is normal. Afterwards, they preferred to track me with MRCP since ERCP is quite difficult process. ERCP was done in this major hospital as well.

As I said, my only symptom was obstructive jaundice in November, 2015 which takes about 3-4 days to be normal, LFT was elevated abnormally : AST: 254, ALT: 764, ALP: 437, GGT: 896, Total Bilirubin 14.72. Beginning of that day, I had very bad day, very upset emotionally because of a family matter. I though this somehow triggered without a concrete clue of course. The only thing I find out, Gilbets syndrome can be triggered by stress and emotional weaknesses if this is my problem.

By considering my genetic background, My mother was removed her gallbladder, has bile acid problem with her stomach from her childhood up to today, couldn’t find out a cure yet.

Ozgun

9

Ozgun,
One things for sure, that bilirubin is out the wall. You must be having a time with itching! I see you mentioned November, 2015. Have you had labs since then? What is your current MELD score? You need to be having labs at least every 6 months with PSC and as your MELD score climbs that needs to be done even more often.

You can check your MELD score by inputting the data from your labs. If you don’t have all these type of labs, ask them to run them for you. Here’s the link to check your score.
https://optn.transplant.hrsa.gov/resources/allocation-calculators/meld-calculator/

Mark

10

Hello Mark,

My latest blood results showed me MELD score as 9. Latest Bilirubin was 2.13 on February, 2018. I keep track my results every 3-6 months.

As said, I now quit taking URSO, will repeat blood test on July, 2018. I still in an uncertain situation for diagnosis of my problem. No strictures on bile ducts were observed on my last 3 MRCP in last 3 years, which is against PSC and my ALP level is normal as well.

Do you or someone from the forum have any knowledge or experience that the liver diseases might confuse doctors with PSC and it is actually another thing at the end? I don’t know how to tell but, the question, what if I am not PSC, what might be my problem by considering I had once an obstructive jaundice in 2015 with elevated LFT in my whole life only as a sign of a problem?

Ozgun

11

Ozgun,
Would you mind sending me a copy of your most recent labs, be sure and mark out any personal information such as your name and address, etc. and post them back on this message. I’ll make an inquiry with one of the hepatologists at Duke to see what they say. Thanks.

Mark

12

Hi Mark,

I send as DM to you, thanks for your kind interest.

Ozgun

13

I received the information Ozgun and have forwarded it on to my hepatologist. Will let you know when I receive a response.

Mark

14

Hi,

I want to update on my case. I don’t use any medicine since 2 years, and newly moved to Brussels. Hereon, I performed MRCP with following outcome (Google Translate):

Conclusions:

Isolated stenosis of the sub-segmental hepatic bile ducts of segment V without any other stigma of cholangitis

Hepatopathy stigma in the form of contoured hepatomegaly

slightly irregular, fatty overload and splenomegaly

So basically, there is still no typical findings for stenosis, my doctor said that the result shows that I am not PSC, but they are wondering about the fatty liver cause (In Turkey they spent years without a firm diagnosis since 14 years, latest point was that I had NASH!..).

My Latest blood results are: Bilirubin: 2.2 ALP: 116, GGT 201, ALT: 87, AST: 45.

They will review the result with radiologists but I think the mystery keeps valid even here. They are thinking to repeat biopsy on my liver which I am not sure about it. I had it 14 years ago with only findings of fatty liver.

Let’s see what they will say.

Ozgun

15

Welcome back, Ozgun.

Glad they did not confirm psc. Hopefully a different diet can help with a fatty liver.
Maybe after that review something will be come definitive.

Jeff

16

Hi Jeff,

Today they return as follows:

We confirm the absence of significant abnormality on your MRI; The best is to perform a new liver biopsy. If is this OK for you, I will contact the “pré-admission” to plan the liver biopsy .

So that I am not sure if I can have liver biopsy again, let’s see.

Regards,

Ozgun

17

Hmm. Never had one, but maybe do a search here to see what other’s experiences have been.