Autoimmune cholangiopathy??

[witchiegirl]

I am still quite unsure what this is. Is it a version of PBC without the ANA? Is it an overlap of AIH and PSC (what I was told initially, but less sure now since reslut of last biopsy). Alll info very welcome,as I know nuffink really!

I did ask the specialist this and a few other things at last clinic and he was quite UNinformative. In fact we had quitea tetchy conversation as I'm not sure he really wanted me to ask questions about my condition - oh dear! Still, I'm being a good girlie and taking the pills as prescribed (at present Prednisolone, which i HATE - but LFTs improving and dose reducing - so I'll persevere)

[witchiegirl]

Of course I meant 'AMA neg' not ANA. Tired brain last night :-). Tiredness not helped by recovering from an attack of shingles. I have ANA, but not AMA. It seems it is overlap of PBC and AIH, sort of what i thought most recently.This does make sense considering other problems I have - great fatigue, nausea (better at mo but there nonetheless), dry eyes, hypothyroid, bladdy bla.

I need to ask (GP, I think, as he is more helpful than specialist) for copy of biopsy result, as I think it said it wasn't PBC, which has confused me somewhat. I got verbal report from GP some weeks ago, on a day i went along with more than one probllem so we didn't talk much about liver disease and the one question I did ask, GP said he didn't know!! (I don't mind doctors not knowing - I prefer it to arrogance and stonewalling). I don't know why this man doesn't want to talk to me about my illness - it should be all in a day's work to him; tis my fecking (shrinking?!) life after all. I think i may ask GP too for lab results? How many should I get - last five perhaps???? Interested to know what others think - I am quite 'neutral' on knowing every last detail, I certainly don't want o make it my life's work to understand my disease - I'd prefer to be well enough to just live my own version of an interesting life, manage my symptoms, and see what happens - though one of my stonewalled questions to the specialist was 'How do you think this disease of mine will progress?', quite reasonable qu in the circs. He didn't really answer that either.....

[Bolly]

Hi Witchie.

I find 2 sources useful when I don't understand something liver related.

One is Google Books.co.uk. Here you find extracts from textbooks, sort of nursing or general practitioner level guides to diseases. I guess the sort of books medical students or graduate nurses would be using.

So for example, type in autoimmune cholangiopathy into the search box within GoogleBooks and you get an extract from Schiff's Diseases of the Liver, which says in a section on autoantibodies and PBC (among other things):

"some studies have reported a high frequency of antinuclear antibodies (ANA) in this group of patients [PBC patients] and have referred to this condition as autoimmune cholangiopathy. However, because the clinical features of the two conditions overlap considerably it is unclear whether these are separate conditions or variants of the same condition with differing autoantibody patterns"

"Clinical Hepatology: Principles and Practice of Hepatobiliary Diseases, Volume 2" [also available to read on GoogleBooks] has a chapter on autoimmune cholangiopathy written by a Henryk Dancygier, His chapter refers to Brunner and Klinge who appear to be the first medics to assign the name to the condition. He has 19 journal references, so you could follow all those up!

My second source is Pub Med online, which brings together abstracts from medical journals. Its difficult to understand a lot of it, as its very technical, but the abstracts are enough to get the gist of who is studying the condition and if they have any clues or answers.

Re your consultant. My view is that its good to have faith/trust in whoever is treating us, and if we don't have that, get a referral elsewhere. I would be looking up your guy on the internet and seeing what his speciality is, he should if he is any good, be publishing papers. If its not PBC or anything related to your condition, go elsewhere. You can use something like Dr Foster to see if you can track down a hepatologist who DOES specialise in autoimmune cholangiopathy and then see about switching. You don't have to be treated by someone within your local health trust, you can go anywhere provided your GP refers you. Of course you have to be prepared to travel if the specialism you are looking for isn't on your doorstep,

Yes a good idea to get the biopsy report. They do seem to vary. My first was done by one hospital, ran to 3 pages and was very detailed. It took me ages to decipher it all.

The second was done by a different hospital and ran to two paragraphs, so not much use in the end! Same liver, same diagnosis ultimately, different protocol.

Blood tests? Ask for as many as they are prepared to give you! It depends how generous and understanding the secretaries are and if they feel your reason for wanting back copies justifies them spending time going back through the data and printing them out. If you get the vibe they are not keen, maybe the last 2 or 3 rather than 5.

Doctors are cagey about giving a prognosis. They can give you the worst case scenario only to find you fit and healthy many years after they said you would not be, or they can say you have a normal life expectancy only to find you getting sicker by the minute. They will probably use statistics of other cases, (if there are any) rather than give you a personal glimpse into the future.

[Bolly]

Here is an example of a medical research journal article. This is Japanese, so presumably been translated. Full article published in the Journal of Gastroenterology:

"Title;Three patients with autoimmune cholangiopathy treated with prednisolone.

Author; MASUMOTO T (Ehime Univ. School Of Medicine, Ehime, Jpn) NINOMIYA T (Ehime Univ. School Of Medicine, Ehime, Jpn) MICHITAKA K (Ehime Univ. School Of Medicine, Ehime, Jpn) HORIIKE N (Ehime Univ. School Of Medicine, Ehime, Jpn) YAMAMOTO K (Ehime Univ. School Of Medicine, Ehime, Jpn) FAZLE AKBAR S M (Ehime Univ. School Of Medicine, Ehime, Jpn) ABE M (Ehime Univ. School Of Medicine, Ehime, Jpn) ONJI M (Ehime Univ. School Of Medicine, Ehime, Jpn)

Journal Title;J Gastroenterol

VOL.33;NO.6;PAGE.909-913(1998)

Pub. Country;Japan

Language;English

Abstract;We describe three patients with autoimmune cholangiopathy, i.e., anti-mitochondrial antibody-negative and anti-nuclear antibody-positive primary biliary cirrhosis, who were treated with prednisolone. Serum anti-mitochondrial antibody and anti-pyruvate dehydrogenase-E2 component antibody were determined by immunofluorescence of frozen sections and enzyme-linked immunosorbent assay, respectively. Immunoblotting using mitochondria prepared from rat liver was performed to analyze anti-mitochondrial antibody in detail. Serum from one patient reacted with a 48-kilodalton protein, but sera from the other two patients failed to react with the mitochondrial proteins. There was a marked improvement in liver function test results after prednisolone treatment. Before treatment liver biopsy in all three patients showed histological features of primary biliary cirrhosis with hepatocellular necrosis. Repeat biopsy during treatment showed marked amelioration of hepatocellular damage in all three patients, although bile duct involvement persisted in two patients. These findings suggest that prednisolone is an effective treatment for hepatocellular damage in patients with autoimmune cholangiopathy, but has little impact on the bile duct involvement. (author abst.)

Final sentence is often the most revealing, so in this case they found that:

"prednisolone is an effective treatment for hepatocellular damage in patients with autoimmune cholangiopathy, but has little impact on the bile duct involvement".

If that was me, I would be asking my hepatologist what treatment there is for the bile ducts as it seems you are on the right treatment for the liver cell damage but prednisolone alone doesn't treat the whole condition. At least according to these Japanese medics.

[witchiegirl]

Thanks so much, Bolly. I have been wondering about my bile ducts involvement too - the recent biopsy showed some mild bridging fibrosis in both my liver and bile ducts, and they seem to be dealing with only the AIH bit at present. mm, I ddi look a few weeks ago for papers by 'my man', found very little really, and was thinking along same lines as you.I will look inot all this again. Cagey does sum up his reaction to my question re ?progression - and I do understand why. i just like facts and then to form my own conclusions. But then his predecessor was probably a bit overoptimistic, so maybe he's being a bit cautious.

I am making heavy weather of steroids at present (lots of side effects AND have had shingles in past week, so will need to find some more oomph before I can do much more, err, fighting, but I can still think and ponder)

I'll have a look at Google Books later. Pub Med I use already. Thanks again.

[Bolly]

Hi witchy. I'm new to this autoimmune malarkey, only started treatment in Feb though I have had viral hepatitis for over 30+ years.

I was put on high dose steroids and am being weaned off them as my hepatologist aims to switch me to an immunosuppressant instead and get me off the prednisolone. I found the pred made me feel sicker than the autoimmune flare. I felt really ill and not able to work for a month. I looked awful too, got the classic moon face and was pale with puffy eyes. Had muscle tremor, agitation, insomnia etc etc. I don't know what the longterm treatment is for PBC but can you take something other than steroids to control the liver damage?

[witchiegirl]

I'm waiting for the right moment to ask the medics 'Is this shit going to actually make me feel better or just make me live longer?'. I HATE prednisolone, though it has to be said, as my dosage goes down (now on 20 mgs per day since Wed. Not counting or anything!) I do feel better - but still having problems with focus (often can't get to end of sentences when speaking - and, no, it isn't just 'perimenopause brain', it's only been this bad since the pred began), sleeping, jitteriness, reading problems, nausea, weird taste (often helped by eating!), craving for sweet stuff which hadn't been happening before prednisolone.Dreadful fatigue, worse than before (which was bad enough) . And the moon face too. I'm really upset to have gained weight - had lost lots, which I needed, and liked. Ghastly mood swings. And I've had shingles, a very mild attack it has to be said, but not pleasant. A few of my other AI problems improved on higher dose (dry eyes, asthma) but back to normal on lower. Oy vay! My skin and hair are luscious. Weird stuff.Lots of weird bleeding - cut myself in kitchen the orher night and took ages longer than usual to stop. My legs couldn't cope with their twice-yearly shave this week - became excoriated, like a huge great graze all down my leg. so i now have half-shaven legs as had to abort the attempt). Lots fo bruising, worse than just with knackered liver.

The plan for me is also to begin azathioprine soon, early June, and they hope to reduce pred further - all depends on bloods. After the first month on pred, my LFTs did go down by several 100 so a good response, and does make it feel worthwhile :-). But only just.

[Bolly]

Fuzziness - brain fog, yes get that too. That's probably the liver damage (toxins in the system) rather than the meds. Mine is no better on a lower pred dose than a high one. Words get jumbled, cant get a sentence out, etc.

Sleep problems, jitters, mood swings - that's the pred.

Food cravings - pred. I was very conscious of the weight issue and was strict with myself. Havent put on a pound but could have eaten more than I did. Appetite back to normal now I'm down to 5mg of pred.

Skin - awful, spotty and blotchy and dry

Dry eyes - have something called Hycosan from the optician which helps a bit.

Fatigue - that's the liver disease, everybody with AIH suffers from it, also those with viral hepatitis. You and I have a double whammy. Rest when you can, listen to your body, change your lifestyle/routine to cope if possible. I work reduced hours now and make sure I eat and rest well between work shifts.

Bleeding/bruising - not the medication. Have you checked your platelet levels?

Yup, pred got my flare (ALT of 660) down pretty quick, back to normal levels in a few weeks, but damn hepatologist wont take me off pred completely as yet.

Been on Aza since 25th Feb and my immune system now shot to pieces - lymphocytes down to an alarming low level means I have nothing to fight a virus with. Neuts ok, they fight bacterial infections.

Review with hepatologist in 10 days

On we plod!