another extraintestinal manifestation - PSC

My dear patients !

To say it right away: the extraintestinal manifestation I write about today is rare. The issue is primary sclerosing cholangitis, the simple abbreviation everybody uses is PSC (nobody has the time and the nerve to pronounce the term word by word).

Even though the name primary sclerosing chonlangitis is actually a sounding and very informative  name. Which tells us exactly what is happening: an inflammation of the bile ducts (cholangitis) which is not caused by external infection (primary: no external reason)  and wich leads to sclerosis of the bilde ducts (sclerosing) over a longer period of time.

PSC (primary sclerosing cholangitis) occurs in average in about 5% of patients with inflammatory bowel disease. In case of ulcerative colitis  in up to 8%, in case of Crohn`s disease in 1 % to maximum 3% of patients. As always data in literature differ a little here, but that`s about the range.

And when you think about the relevance of this extraintestinal manifestation in case of inflammatory bowel disease in general and ulcerative colitis in particular, you kind of have to turn the tables. Because PSC  (primary sclerosing cholangitis) in case of inflammatory bowel disease is rare. BUT from all the patients with PSC about 70% develop or already have an inflammatory bowel disease, mostly ulcerative colitis.

And maybe theres is the one or the other of you who reads this thinking: “That`s exactly how it was in my case. First there were the elevated blood values showing there was something going on with my biliary system which turned out to be PSC. And it was only years later the gut was becoming a problem and ulcerative colitis was diagnosed.”

Primary sclerosing cholangitis 8or PSC) belongs to that kind of extraintestinal manifestations which can occur even years before the inflammatory bowel disease. Years before you even recognize somethings going on in your stomach or your gut. Or even years after it.

And I wrote it in the article with the common information on the EIM (extraintestinal manifestation): even after colectomy in case of ulcerative colitis PSC can still be active or even just develop.

I can´t say it any differently, it actually is reacherous or at least can be.

Ok, until now it was a little statistics here. 

Who get`s it.., when does it occur ? 

But what exactly is happening in case of primary sclerosing cholangitis , PSC ?

what happens in case of PSC ?

So PSC (primary sclersoing cholangitis) = inflammation of the bile ducts.

Just like that ?

The assumption is, that small parts of the intestinal content – we call antigens – or  inflammatory cells and cells of the immune system migrate from the bowel to the liver where the bile duct system is located and just do what they would have done in the bowel – cause inflammation. Because that`s just what they do. Broadly speaking.

When one realizes that actually the bloodflow from the bowel goes straight to the liver (exactly – where the biliary system is located) the fact that inflammation from the bowel can “jump over” to the liver even gets conceivable in a way.

Inflammation of the bile ducts in case of PSC (primary sclerosing cholangitis)  gets chronic like in inflammatory bowel disease. Which means that it is not an inflammation as in the case of pneumonia for instance which occurs – and passes under therapy.

It is rather an inflammation that stays or recurs.

And as in the case of inflammatory bowel disease Crohn`s disease and ulcerative colitis  this inflammation of the bile ducts isn`t active on the same level all the time. It is changing and can even show just a little or no activity at all for a shorter or longer period of time. 

And it rarely does affect the whole liver at the same time and in the same extent either. Mostly some parts are more affected than others. 

In the further course – after many years or better: after recurring inflammation – the result is fibrosisis of the bile ducts.

And that´s what causes the problems. After recurring inflammation the bile ducts are not soft ond flimsy anymore but get scarred and rigid. The bile can not flow freely from the different parts of the liver anymore.

Sometimes a stricture or stenosis forms you can actually detect in the diagnostics, which can lead to smaller or larger bile congestions. Sometimes there are “just” (bad enough actually) some irregularities of the bile ducts which lead to recurring inflammation as well, even though there is no detecable congestion.

And since the bile ducts are located inside the liver, the liver is of course affected by the inflammatory process. 

the bile duct system inside the liver...

At that point I realized, that some of you might not even know so much about the bile, the biliary system. Where the bile ducts are and what they are even good for. Most people know the gall bladder. Which doesn`t play a role for primary sclerosing cholangitis, PSC.

Some might have heard  about the large bile duct in the context that gallstones had to be removed from there.

So I will write here very short how things work with the bile duct system inside the liver. 

Bile is produced by the liver cells and in the first instance it is transported in very small, for the naked eye invisible ducts between the liver cells. These small ducts you really can only see through the microskope. But it is the same principle as in other parts of the body: small vessels  unite to bigger ones. The bigger ones finally unite to even more bigger ones and in case of the biliary tract finally two ducts unite to the large bile duct (make no mistake, large means 4 mm here). In which the bile is transported from the liver to the upper intestinal bowel, the duodneum to be more exact here.

The gall bladder is just – in the sense of exclusively – a storage, it is not essential and as I said it doesn`t actually play a role for PSC.

A problem of the bile duct system can result in a problem of the liver

And I write it so epicly for making it clear that a problem of the biliary tract can result in a problem of the liver. Because when the small bile ducts or the larger ones show inflammation and fibrosis and that  results in irregularities and final stenosis of the bile ducts – es described above – the result ist a bile congestion in the liver. Which leads to inflammation of the liver and changes the structure of the organ.

Which means fibrosis as a result of inflammation turning into cirrhosis at one point.

At least some of you have heard about liver cirrhosis and know that it means loss of liver function. And now the circle is completing to the point where I mentioned that primary sclerosing cholangitis, PSC can have far-reaching consequences. Which means: some of the patients with PSC (primary sclersoing cholangitis) need to  undergo liver-transplantation, because that´s the only way to stop the increasing decline of liver function. Liver-transplantation is a radical and indeed far-reaching aspect. Not all patients with PSC have to undergo transplantation. I will write more about the therapy in the next article.

Today I wanted to share information what exactly happens in case of PSC (primary sclerosing cholangitis… everybody is using the abbreviation right ? I wrote the long complicated term all the time and you recognized it, I know. But I bet now you recognize the term everywhere, wether it`s in a doctors report, in an article or when you talk to a doctor or hear doctors talk. And you know what it`s about and what is going on. 1:0 for you)

So again: PSC (primary sclerosing cholangitis) is a rare but important extraintestinal manifestaion of the inflammatory bowel diseases crohn`s disease and ulcerative colitis.

How you recognize an PSC and what kind of diagnostics need to be performed and what we can do about it you can read in the next article.

 

For today take care and best regards,

 

Dr. Susanne Weyrauch

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Warum?

And at least some of you have heard about liver cirrhosis and know that it means loss of function. And now the circle is completing to the point where I meintioned that the PSC can have far-reaching consequences. And that means : some of the patients with PSC (primary sclersoing cholangitis) need to  undergo liver-transplantation, because thats the only way to cure the disease.

wich is a radical aspect, especially since a lot of young people are affected with IBD, who have years  and decades with the disaese and the EIM

 

So again: PSC is a rare but improtant extraintestinal manifestaion of the inflammatory bowel diseases crohn`s disease and ulcerative colitis.

 

How you recognize an PSC and what kind of diagnostics need to be performed and what we can do about it you can read next week.

 

For today take care and best regards,

 

Dr. Susanne Weyrauch

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Watch your eyes…EIM of the eyes in IBD

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