Could anyone explain what what the various stages are as I haven't a clue.
The letter diagnosing me from the specialist was vague at best. It just says that the gamma GT is 38 just above the normal 35 and igM was marginally elevated. In her words "I therefore guess we do have the diagnostic criteria of early PBC .
So many questions.
Thank you.
Copied from a online article.
Stage 1 (portal stage of Ludwig): Portal inflammation, bile duct abnormalities, or both are present
Stage 2 (periportal stage): Periportal fibrosis is present, with or without periportal inflammation or prominent enlargement of the portal tracts with seemingly intact, newly formed limiting plates
Stage 3 (septal stage): Septal fibrosis with active inflammatory, passive paucicellular septa, or both are present
Stage 4 (cirrhosis): Nodules with various degrees of inflammation are present
Hi Jules -S,
The 'stages' of PBC are the actual stages that it goes through once PBC has absolutely been confirmed and diagnosed. From what I can remember, the stages that ranrace describes are accurate, but even the first stage of PBC cannot be accurately diagnosed without a liver biopsy. You don't indicate that you have had a liver biopsy.
Your post, so far, seems to suggest that you have only been examined by a specialist, and that you have had blood tests done. It is good that they are being careful, but I think it is early days and more tests are needed - I think that with just the readings you mention a lot of other things could be going on. It's always possible that other tests have been done, but you need to ask for a full printout of all your blood test results and full reports of all examinations [I'm in the UK, and it may be different for you if you live elsewhere, but my understanding is that in the UK we have a 'right' to a full copy of all test results and examn findings].
Also, is you specialist a liver consultant - specifically a PBC specialist?
Obviously specialists need to look beyond the strict guidelines, and take into account all worrying results, but: you don't mention if you have been tested for AMAs (antimitochondrial antibodies) ... most people with PBC do have these, although it, alone, is not a definitive test for PBC, and about 5% of people can have PBC without testing +ve for AMAs. Plus it would be interesting to see all of the other liver function tests: there are a bout a dozen, and as well as GGT and igm levels, ALP, AST, ALT and others are important.
Finally you don't mention symptoms. You cannot be diagnosed with PBC just on symptoms alone - as lots of other liver conditions, as well as other autoimmune conditions and illnesses can have these symptoms, but they help to point specialists in the right direction for further tests. Also, you don't say if you have had an ultrasound of the liver, pancreas, spleen, gallbladder, as checking on all these helps.
Strictly, PBC is diagnosed on the presence of 2 out of 3 of the following 'diagnostic criteria':
1 - the presence of AMAs; and / or:
2 - abnormal (blood) liver function tests [eg the GGT, though that's not very high: 40 is 'normal' at my specialists lab] which are typical of PBC; these are usually monitored over a period of months: and/or:
3 - a biopsy of the liver that shows damage to the bile ducts, that is typical of the abnormalities associated with PBC. [this is what ranrace mentions as 'Stage 1'].
You really need 1) and 2) for a formal diagnosis; 3) is often done if 1 or 2 are absent, but it depends on the symptoms, on further tests (you should be checked for all sort of other autoimmune and liver conditions) and on quite how the liver blood tests are abnormal.
Sorry this is so long, but I think you need a lot more info, details and results and guidance from your specialist. I would talk to the 'PBC Foundation' [link at the top of this page], they host this site on 'Health Unlocked' . Their advisors are great, you can email or phone, but I think it would help if you have all your results and medical history to hand.
Hope this helps. Take care.
Please don't apologise for the length of the reply, I'm really grateful and you have given me a lot to think about. I do live in the Uk so will ask for a copy of my blood test results. I have seen a liver specialist not a PBC specialist. I have had two types do scans, ultrasound and another type which felt like shock waves being fired!? Both of which said my liver was in really good condition, I just had a bike duct which was slightly dilated, but I was told this could have been caused by passing a stone. There is no mention of AMA's in the vague letter just LgM being marginally elevated. As for symptoms before I started taking urso I was very tired with painful joints throughout my body. I have an appointment with rheumatology in a few weeks. I can't help feeling she rushed into the diagnosis. Thanks again, when I get my new blood results this week I will contact the PBC foundation advisors.
Even now, a lot of liver specialists may not have had to treat a patient with PBC, and don't always know or follow the strict procedures. If you read around on this site, you will find loads of people who came to their diagnosis - or their 'all-clear' by all sorts of different routes.
I only have AMAs, I may have always had them, but when they were spotted 24 years ago, I wasn't told anything scary, as all other tests were clear. The rheumatologist and hepatologist I saw both said I no conditions (eg PBC or rheumatoid arthritis but these were never mentioned) & my GP told me to have blood tests every year, so he kept an eye on things - I never declared any illness (to Insurance). Years later, when I mentioned AMAs to a new GP, she panicked, did tests, told me I'd got PBC (!), and sent me to a liver consultant! He said I hadn't got it, but to keep up annual blood tests ... but when his letter came he'd 'diagnosed' me as having 'pre-symptomatic PBC'. This is not a recognised diagnosis and played havoc when I innocently mentioned it to insurance - they insisted on treating me as if I had PBC.
After years of no symptoms, and 'perfect' annual liver function tests (= the GGT, ALP etc) I demanded to see a leading PBC specialist. My GP agreed, and I saw one of the best guys in the UK. He says I don't have PBC, and it was also him who told me not to worry about the AMAs, as about 10% of the population have them, and most never get PBC ... and also he told me what the strict 3 diagnostic criteria are. Obviously these 3 criteria are not all that they go by, as PBC has many faces, but he had also checked all my medical history, as well as my family's health history, and he felt I was not at risk.
But it really does depend what it says on your full results. The specialist should have sent them to your GP, and I always just ask my GP's receptionist to print the tests for me when they've been done. I would ask your GP receptionist: choose a good time - and the nicest one there - and with luck they will print them out for you; there may be a small copying charge. I don't know much about the igm - is it an inflammatory marker? I guess if they are also worried about joints, that might be up. But then if urso has eased the tiredness, maybe they are wise to consider the PBC possibility - I don't know, but don't want to raise your hopes. Certainly ultrasounds, fibroscans, etc cannot actually diagnose PBC (only a biopsy can do that): they show obvious scarring or fatty liver, so that maybe means you don't have other issues.
I hope you get some answers, and please do let the community here know what happens, as it is always helpful to hear more about PBC and not-PBC.
Meanwhile, try to have fun and do things you like. All autoimmune conditions thrive on stress, so coddle and treat yourself. PBC is generally not so bad now, and there are also new treatments in the pipeline. Take care.
Gritty xx
Hello Jules-S.
Neither have I either. Quite frankly I am not interested in these so-called stages of PBC. I was diagnosed with PBC due to symptons (itching and fatigue in 2010) and abnormal bloods and then it was found I had a high titre of antibodies specific in PBC so for me being in the UK no biopsy was recommended.
Although PBC can be diagnosed via a biopsy which is apparently due to cell changes that are throughout the liver, I think with the liver being a large organ, the biopsy site might not necessarily show a true picture of how it really is.
I go with how I feel. I am not so sure regarding the bloods either as once we are taking urso I do think they can be cheated on a bit with taking this. Some taking urso state they go back to within normal range for their bloods, others never do though it is said to be fine with PBC (mine have never reverted back to normal readings on urso but aren't that bad).
I think this staging is individual. I am quite confident that standard ultrasound can show how the liver is doing. Saw a programme on tv pre-diagnosis and 2 guys were showing variable conditions, one with fatty liver, the other cirrhosis due to him being alcoholic.
Hi Peridot. I fully agree with what you said. I do not know the Stage and is not interested to know more. I just try and be positive, knowing there is no cure and the itch is part of my life. There are people that is still worse off than we. (Easier said than done, but it is the only way I can handle this PBC) I take my Ursotan, and eat very healthy, cut gluten out. I know this autoimmune have attacked other areas, battle with my teeth, dry eyes and mouth, eczema on hands etc. Glad I became part of this group.
Hi Jules-S,
I live in the USA and was diagnosed with PBC and Overlapping Autoimmune Hepatitis this Feb 2016. I work for a Rheumatologist. I had very high blood levels Alkaline Phosphate, ALT AST ( all associated with liver) I also had Ultrasound of liver, gall bladder. Then followed up with a Liver Biopsy. I was covered in rash head to toe. The Rheumatologist will test you for all kinds of Autoimmune diseases that share same kinds of symptoms. Those shared symptoms can be conflicting and confusing. I'm happy to hear you are seeing a Rheumatologist. The URSO is used for the bile ducts that have been plugged to break up the bile, which in turn causes the skin to itch from overflowing bile into the bloodstream. I am also taking Imuran for the overlapping autoimmune hep. Crazy how your own immune system decides to be a traitor and attack itself. haha! I work full 9 hr or longer days running a medical company. I am exhausted at the end of the day but happy to be alive. I stay to busy helping staff and others i don't have time to fret about myself. Apparently, I have had this disease for many years but its was not as common here in the USA. I have Celiac Disease on top of everything else since 2008. I recently cut out meats, started eating very clean diet and eating vegetarian. This week introduced fish back into my diet. I have felt so much better and lost 7 lbs. I had gained loads from being on Prednisone 40mg for months. They stuff almost killed me but luckily my boss (the Rheumatologist) contacted my Liver dr said he was tapering me down because i couldn't barely breath after walking from car into the office. You will read so many different stories of everyone's experiences but PBC seems to take affect differently in each of us and our mental state has lots to do with our carrying on to live happily. I wish you the very best. I hope your Rheum doc does all the proper tests to eliminate different autoimmune diseases that can make you feel achy, sore joints, tired, and much more.
Theresa
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