PSC II - Symptoms, Diagnostics, Therapy

My dear patients !

So how do you actually recognize, wether you have PSCprimary sclerosing cholangitis or not ?

The good thing is: in the first instance you won`t even recognize it. And I write “the good thing” because in the beginning of PSC, the primary sclerosing cholangitis you won`t have any symptoms or discomfort.

By the time symptoms occur there are changes in the bile duct system already. If the bile flow in the liver is impaired it results in inflammation even when just a small part is affected.

Sometimes we can`t even detect the obstacle and the constipation in diagnostics (I will write about that below). Sometimes a small irregularity in the wall of a single bile duct is enough to cause inflammation.

But what do you actually recognize ? 

It is rather unspecific symptoms like feeling weak and tired.You might have slightly elevated temperature (38°C or 38,5 °C), maybe sweating at night.

The one symptom that is often described in case of PSC , primary sclerosing cholangitis is – a really annoying – itching of the skin, for which the cause remains still unclear.

This itching can rather occur without remarkeble laboratory findings or without detectable bile congestion.

In case of bile congestion the white part of the eyes and the skin turn yellow (we talk about “icterus” then, which means jaundice). That does definetly not belong to the “early smptoms” category but to the “go see a doctor! hurry!” category. 

first it`s just elevated laboratory values...

In the beginning of PSC, primary sclerosing cholangitis there are just elevated laboratory findings which indicate cholestasis.

I do not write “liver function  tests” on purpose. And I really want to make the point what blood values are important in case of PSC, primary sclerosing cholangitis so there won`t be confusion or trouble for wrong reasons.

It is the ALP that is the important blood value. ALP stands for alcaline phosphatase (for the sake of completeness),

The second important blood value is the gGT (gamma-GT, stands for Gamma-Glutamyltransferase) .

These parameters (AP and gGT) are also known as parameters forcholestasis (…I already wrote it above. What a word again ! Some of you might think: “enough ! stop the weired terms now Dr. Weyrauch!” But do you know what ? The term cholestasis is used so often. And I am pretty good in avoiding specialist vocabulary while talking to my patients. But cholestasis or parameters for cholestasis (which means ALP and gGT) are really used very often. You will hear it very often and as always – good for you when you  recognize the term and know what it is about)

And that these blood values for cholestasis are elevated is something your doctor should recognize

The liver function tests -which mean all values relevant for the liver- start to increase, when the liver tissue is already affected by PSC. (When the liver gets affected in case of primary sclerosing cholangitis you can actually read in another article on PSC). It is rather rare in the beginning of the disease.

It is necessary to have an eye on the AP and gGT  every once in a while anyway. Because being a patient with IBD, inflammatory bowel disease, Crohn`s disease or ulcerative colitis you might be on medication which can affect these values as well.

(To be quite honest: I do not know of a single medication which is not able to cause an elevation of these parameters.)

And also in changes of these parameters caused by medication it is often the AP and gGT that are elevated.

Which leads us to the question: when AP and gGT are increased in case of PSC and are increased as a result of medicatin intake – how can we probably recognize what exactly is the cause for the elevation ?

And that is exactly the right question !

Because it is not easy to distinguish either.

I always handle it that way: whenever a patient with inflammatory bowel disease, Crohn`s disease or ulcerative colitis  and especially a patient with ulcerative colitis shows elevated values of AP and gGT, continuously (8 weeks) and without good explanation (medication isn`t a good explanation. It is an explanation but just when all the other things and especially PSC have been excluded) the possibility of primary sclerosing cholangitis is emerging and needs to be taken care of. How this is happening I will write below

And in diagnostics for PSC the trick is not to perform diagnostics once but eventually repeat it. When I suppose PSC in a 25 year old patient and diagnostics do not confirm PSC, it doesn`t mean that it won`t be necessary to repeat the exactly same diagnostic procedures again after 1 or 2 years when I still have the strong suspect of primary sclerosing cholangitis

the diagnosis PSC ...

Diagnosis of PSC (primary sclerosing cholangitis) is confirmed , when there are typical laboratory findings and evidence of bile duct irregularities.

For that MRCP is performed. 

An MRCP is an MRI of the liver, the bile duct system and the pancreas with a special contrats media, which gives a very good image of the bile duct system. 

When results of that examination do not allow to confirm the diagnosis primary sclerosing cholangitis  but you as a doctor are sure it is PSC, ERCP should be performed.

Which is an endoskopic procedure that is performed with a special endoskope that is placed in the part of the upper small intestine (the duodenum to be more exact, located directly after the stomach and good accessible) where the main bile duct enters the small intestine.

During the examination the endoskope is introduced up to the point where the large bile duct merges into the upper small intestine (duodenum). And over this endoscope the large bile duct is probed with tiny wires and finally the bile duct is contratsed and screened.

You perform ERCP when you see bile duct irregularities in the MRCP intent to widen stenosis or to place stents in a stenosis.

Or you can do diagnostics by collecting a cell sample with a small brush from the bile ducts.

Why that ? Why have a cell sample from tha bile duct wall with a little brush ?

Because statistically the risk for carcinoma of the bile ducts or the gall bladder is elevated in case of  primary sclerosing cholangitis.

So do you actually want to know what MRCP stands for ? Or ERCP?

I recognize myself that the issue of PSC, primary sclerosing cholangitis has a lot of skurrile terms, it kind of gets out of hand ecspcially in combination with the weired abbreviations. 

But I promise: nobody, really nobody, not even the most hard boiled colleague is pronouncing the whole terms. The abbreviations ERCP and MRCP are sufficient. As is the abbreviation PSC.

I will write the complete terms for the sake of completeness. Those who really don`t want to bother with weired terms just skip the next section.

The other ones take a deep breath: MRCP = magnet resonance cholangio pancreaticographie. Ok ? Take another deep breath… ERCP = endoscopic retrograd cholangio pancraeticographie

Well… as with the term PSC the acutally beautyfull thing is that the terms again tell us exactly what is going on. They are not bloated word cases, the terms let you know what the examinations are about.

Let`s stick with the abbreviations though.

Ok so for diagnostics: 1. recognizing laboratory values, 2. do investagation by performing MRCP or ERCP. Always MRCP first, non invasive diagnostics always first.

What else ? – Colonoscopy !

What ? Why colonoscopy ? We were talking about the bile duct system, right ? 

Wich is true, but in case of primary sclerosing cholangoitis (PSC) there is statistically an elevated risk for developing colon cancer. And that`s why from the point where PSC is diagnosed colonoscopy once a year is strongly recommended.

… I always try to take my patients point of view when I talk to them or explain something to them.

And I know that what I write here is quite something.

Not the diagnostics I wrote about. But above it says: elevated risk for carcinoma of the bile ducts or the gall bladder. 

Then it says: relevant risk for colon cancer…

I would love to write something different, but it`s part of the issue of PSC.  And I would actually not take you seriously  if I didn´t share all the information but just the “not so ugly-ones” . That it sounds different than the part about nutrition is naturally clear of course.  

So before I will write about the therapy there is one thing I really want to emphazise: 

There is no: “whenever there is… then there is …!”

Not in case of PSC, not in case of Crohn`s disease, not in case of ulcerativ colitis, especially not in case of inflammatory bowel disease anyway. 

And therefor please do not be discourages wether PSC was diagnosed years ago  or wether the diagnosis has just been established for you.

There is no: whoever is diagnosed with PSC will have carcinoma of the bile duct system. Or :whoever has PSC and ulcerative colitis will get colon carcinoma. Or: whoever has PSC will undergo liver transplantation

What can we do ?

So now what is most important to me. It is always important to me to be able to offer something to the patients or at least to provide a perspective. Because mostly there is one.

First : Therapy of primary sclerosing cholangitis (PSC) has nothing to do with therapy of your inflammatory bowel disease. There has been a lot of research and there still is wether the one or the other antibody therapy won`t better symptoms or laboratory findigs of PSC.  There are interesting approaches if certain inflammatory cells might be influenced  bei antibody therapy in the gut as well as in the liver respectively the bile duct system. But data are (yet ?) not like that we could  assume that patients with PSC really benefit from antibody therapy.

And that`s why everything stays the same.

The same… good old ursodeoxycholic acid. Abbreviation: UDCA  (again another weired term. I`m telling you, the issue PSC is sophisticated on various levels, just looking at the terms describing different aspects of the disease)

This ursodeoxychic acid, UDCA is … well, it`s the best we have so far for primary sclerosing cholangitis, PSC.

It`s not a therapy. Even when it is listed under therapies. Therapy does cure or delay in a relevant way or it even improves. Definetly. Clear advantage over placebo. Which is right for UDCA … in a way. It is assumed that by influencing the laboratory parameters in a positive way the course of disease might be influenced in a positive way either. When you read through the literature it drives you kind of crazy. Because everybody is always listing everything: the trials which showed an effect and the trials that didn`t … It has been demonstrated that UDCA has a positive effect on the risk of getting colonic cancer. It has been shown that it doesn`t either. And there has been research  where it seemed like ursodeoxychoic acid doesn`t change anything, doesn`t improve anything. There were even trials in which ursodeoxycholic acid, UDCA even impaired the situation. Everything is in there. The different results in different trials had their cause partly in different dosages. Dosage recommended for primary sclerosing cholangitis  is 10-15 mg / kg / day. And in that dosage ursodeoxycholic acid seems to stabilize laboratory values in case of PSC.

A new approach which is not (yet ?) available for standart therapy but is only used in trials with good results so far is Nor-UDCA.

What else can we do ?

I wrote before that there is recurring inflammation in the bile ducts. In that case antibiotics are the therapy of choice since they  are very effective. Because the reason for inflammation is at least in part the closeness of the liver and bile duct system to the bowel and its bacteria. And in case of inflammation because of bacteria antibiotics help excellently.

I wrote about ERCP. When we detect stenosis in MRCP there is the possibilty of widening the stenosis with ERCPI am enthusiastic about this method since I am a gastroenterologist. Its a great technique and it works excellently. Widening stenosis is a good option. Sometimes you place littel stents as placeholders, which can improve and secure bile flow in the liver.

And then there is  liver transplantation.

Well that is – and I said it before – radical. But sometimes it s the only possibility left.

I don`t want to write about liver tarnsplantation here in detail, because it  is really not an IBD (inflammatory bowel disease) issue. Patients with active primary sclerosing cholangitis who show an impaired liver function should be transferred to a specialized hospital which are mostly university clinics. They should be seen by the specialists who can decide what examniations are necessary and wether or when it´s time for listing for transplantation. 

puh…

Yes, this article turned out longer than I planed it. And I do recognize what I have written here:

  1. you won´t recognize it
  2. when you recognize it it`s not the beginning anymore
  3. There are good diagnostic methods to establish the diagnosis
  4. there is an increased risk for no less than two kinds of malignacies
  5. When diagnosis is established there is no effective therapy but just a medication that we think might do some good
  6. liver transplantation

But I am convinced that as a mature patient you may and should and actually want to know what we as doctors know about your disease. Because it is your concern. 

And information can contribute to your security because uncertainty decreases and you know what you can expect.

I think who ever searches for and gets information here on I.M. INFORMATION MATTERS has a similar point of view.

I feel like promising to write more easy going the next time. I will think about something.

For today take care !

Best regards

Dr. med. Susanne Weyrauch

RELATED TOPICS

EIM of the skin

EIm of the skin My dear patients  ! And why not continue with the extraintestinal manifestations of the skin ?  Also the skin and the mucosa (the oral mucosa to

Watch your eyes…EIM of the eyes in IBD

Watch your eyes - extraintestinal manifestation of the eyes in case of ibd My dear patients  ! To have many ideas is nice, but when it ends up in starting

Relax…!

Relax ! My dear patients ! Relax ! I am serious. As simple and even trivial this stereotyped phrase sounds, I am dead serious.  Please ensure to actively and consciously

The microbiom – it makes a difference …

The Microbiom - it makes a difference... My dear patients ! The last article focused on how stress can affect the course of your inflammatory bowel disease, your Crohn`s disease

Stressed out ! – what happens in the intestine ?

... Stressed out - what happens in the intestine ? My dear patients,stress remains at the top of the  list when it comes to answering the question: What modifies the

Die Therapie

RELATED TOPICS

EIM of the skin

EIm of the skin My dear patients  ! And why not continue with the extraintestinal manifestations of the skin ?  Also the skin and the mucosa (the oral mucosa to

Watch your eyes…EIM of the eyes in IBD

Watch your eyes - extraintestinal manifestation of the eyes in case of ibd My dear patients  ! To have many ideas is nice, but when it ends up in starting

Relax…!

Relax ! My dear patients ! Relax ! I am serious. As simple and even trivial this stereotyped phrase sounds, I am dead serious.  Please ensure to actively and consciously

The microbiom – it makes a difference …

The Microbiom - it makes a difference... My dear patients ! The last article focused on how stress can affect the course of your inflammatory bowel disease, your Crohn`s disease

Stressed out ! – what happens in the intestine ?

... Stressed out - what happens in the intestine ? My dear patients,stress remains at the top of the  list when it comes to answering the question: What modifies the