My wife has had Fibromyalgia, Sjogren's and Reynauds for about 12 years. Apparently she contracted these autoimmune conditions after undergoing routine rotator cuff surgery. In the last few weeks, she had blood work done in connection with a colonoscopy. Her gastroenterologist order a liver panel that came back with an M2 reading of 126.4 and an Actin smooth antibody level of 30. The other levels were negative. After ordering a liver biopsy the results came back 2 days ago with a diagnosis of Primary Biliary Cholangitis. Specifically, the biospy was negative for the iron stain but the "Special Trichrome Stain" was positive for portal fibrosis and the "Special Stain for Reticulin" was positive. Her physician started her on Urso (250mg 3xday) but no one has really provided us with any real information about next steps or the prognosis. The only real information I can glean from the biopsy and pathology report is that she either has (or had) PBC, ie. another autoimmune condition for at least 8 or 9 years (disguised as Sjogren's) and that it is now in the process of going from asymptomatic to symptomatic. I'm not sure about the options of liver transplant, whether we should even be concerned about it at this point or whether we should stay in a "holding pattern" and wait and watch what develops. I'm not the kind of person who is "reactive". I'm more of a proactive person. Can anyone give me any guidance on what they've do or would suggest to address this issue before it's too late? Thanks
Stages of PBC-Prognosis-Next Steps? - PBC Foundation
Stages of PBC-Prognosis-Next Steps?
If pbc has been confirmed & the doctor doesn’t seem like he/she is engaged in helping with its management, would suggest that your wife go see a hepatologist, a doctor who specializes in liver conditions.
The hepatologist can give you more in depth information on pbc & your wife’s specific case in the context of progression. At the appointment with the hepatologist, have all the relevant medical history available so that the doctor can review & assess. The hepatologist can then manage your wife’s condition.
Good luck.
Thanks. That's exactly what we've done. We've got all of the CDs with medical records and have an appointment with a hepatologist for 5/14.
Just when I thought we had her autoimmune conditions under control, after 12 years (10 years of which involved doctors prescribing opiates to manage symptoms (she's been "opiate-free" since March 2017), a subtotal colectomy and reverse ilieostomy), PBC now shows up.
In short, this really sucks.
So sorry for your wife. This sucks big time but all we can do is make sure we get the best medical attention that we can & be demanding & ask a lot of questions. Feels better when we are in control.
Keep us posted!
Will do. Interestingly, I've had to move from New Jersey to California to Florida and now our appointment with the hepatologist is in New York City. Going full circle.
There are world class hepatologists in NYC especially if they are in the teaching hospitals, leading edge care. Your wife will be fine!
Thanks for the encouragement. It's appreciated.
I have been diagnosed with PBC in November and am currently being treated/ monitored at UCLA phleger liver institute - how about moving back to CA?
Sickamo, sorry for the delayed response. All of our children either live overseas or live in the Northeast. While UCLA may be an option we're going to start in the New York area because we have friends and relatives at Mount Sinai Hospital who are well-connected enough to follow up on my wife's conditons if needed. If I feel we're not making any progress we will, of course, consider relocating to California. Hope your treatment goes well. Get better soon.
In my situation, my PCP was also a GI & he sent me to a hepatologist for diagnosis. My PCP spoke to the hepatologist & provided him with my medical history before my appointment with the hepatologist.
Since then, I follow up with my hepatologist quarterly & I basically let him manage this condition on my behalf. I let my PCP know what is going on at my annual physical.
Hope this helps.
Thanks for the advice and input
Portal Fibrosis I believe is the first stage of PBC which would be good as she is now on Ursu which can slow the progression.
Histologically, PBC is diagnosed into 4 stages with the first 3 stages being considered Fibrosis and the 4th stage being Cirrhosis. Then once you hit stage four Cirrhosis, there are 4 additional subsets:
Stage I Fibrosis is referred to as the florid duct lesion or nonsuppurative destructive cholangitis and is characterized by mononuclear inflammatory cells surrounding a small bile duct. Inflammation, abnormal connective tissue, or both, are confined to the portal areas.
In stage II Fibrosis is proliferation of the small bile ductules. Inflammation, fibrosis, or both, which are confined to the portal and periportal areas.
Stage III Fibrosis is characterized by bridging fibrosis or scarring. Bridging fibrosis refers to the presence of fibrosis that reaches from a portal area to another portal areas.
Stage IV is Cirrhosis which consists of extensive bridging fibrosis in the presence of regeneration such that normal portal areas and normal central veins cannot be identified
These histological stages demonstrate the progression of the disease from destruction of the intrahepatic bile duct to fibrosis and then cirrhosis.
Cirrhosis is stage 4 of pbc & is a subset of pbc progression. Within the cirrhosis condition are stages to describe its particular progression. The 4-stage system is based on the presence or absence of specific complications of cirrhosis. These complications develop because of failing liver function and/or blockage of blood flow through the liver caused by extensive scarring within the organ.
Stage 1 Cirrhosis is the earliest stage of cirrhosis and is characterized by the absence of two significant complications known as varices and ascites. Varices are dilated, ballooned veins. They are most often located in the lining of the esophagus and/or stomach. Acsites refers to an accumulation of fluid in the abdominal cavity.
Both varices and ascites develop primarily because of obstructed blood flow through the liver, a condition known as portal hypertension. While people with stage 1 cirrhosis have extensive liver scarring, it is not severe enough to cause substantial portal hypertension and its complications.
Stage 2 Cirrhosis is marked by the development of esophageal varices, due to worsening portal hypertension, but without the presence of ascites. While the development of esophageal varices indicates worsening cirrhosis and an increased risk of dying in the next 12 months, stage 2 cirrhosis is still considered compensated cirrhosis. There remains the potential for at least partial reversal of liver damage if the underlying cause of cirrhosis is eliminated or cured.
Stage 3 Cirrhosis is marked by the development of ascites, with or without the presence of varices. The volume of ascites varies from being detectable only with imaging tests, such as abdominal ultrasound, to obvious bloating of the abdomen. The development of ascites signals worsening portal hypertension due to advancing liver scarring and deterioration of liver function. Stage 3 cirrhosis signals decompensated cirrhosis, meaning the liver is failing. Once decompensated cirrhosis develops, liver scarring is irreversible and evaluation for liver transplantation is generally recommended. A variety of signs and symptoms may be present with stage 3 cirrhosis, including:
-pale and/or yellowish skin
-weight loss and loss of appetite
-shortness of breath
-extreme fatigue
-persistent, widespread itchiness
-swelling of the feet, ankles and lower legs
-wasting of the muscles of the arms and legs
Stage 4 Cirrhosis involves gastrointestinal bleeding, usually from ruptured varices in the esophagus or stomach. This type of bleeding can be immediately life threatening if not controlled. Even if bleeding stops or is medically controlled, however, individuals with stage 4 cirrhosis still face a high risk of dying within 12 months. Persons with stage 4 cirrhosis have end-stage liver disease and urgent evaluation for possible liver transplantation is necessary. Signs and symptoms that might develop include those that may occur with stage 3 cirrhosis as well as others, such as:
-confusion, personality changes and/or extreme sleepiness
-hand tremors
-reduced urination, which may indicate kidney failure
-high fever, signalling infection of the abdominal cavity
Thank you for the comprehensive education Ghosthunter. I'm hoping she's in Stage 1. We look forward to receiving confirmation from the hepatologist when we visit her on 5/14. However, given the extensive history my wife has been through and the overlapping symptoms from Sjogren's and Fibromyalgia we're also realistic and prepared for a less than optimal diagnosis. Having been where we've been every day she wakes up is a blessing.
All any of us can do is to eat healthy, take our meds, try to stay positive, and see our liver doctor on a regulat basis. We are all in God’s hands!
Usually the biopsy can indicate the stage of the condition. Have the hepatologist interpret the biopsy results for you in layman’s terms.
A liver sonogram can also check on any physical anamolies. Lastly, a fibroscan can measure the elasticity or stiffness of the liver.
These are all tools that the hepatologist can use to assess progression of pbc along with the comprehensive metabolic panel of the blood work.
Thanks for the insight. I’m sure we’ll both feel more comfortable after the visit with the hepatologist
Sorry to hear about your wife. Most people do react well to the ursodeoxycholic acid tablets. After a few months all the main elevated liver function test results will rapidly decrease. Urso is very good at holding the disease at bay for many many years. Also there are very few if any side effects of urso. We do all feel fatigued and most suffer from brain fog. The important thing is to have regular blood tests every six months. Also to have a non invasive fibrosis test which directs a sound wave to the liver and measures the rigidity of the liver. It’s a new test and the best one for identifying the stage of the disease.
Thanks. Looking forward to learning more about the specifics of my wife’s condition when we meet the hepatologist on the 14th (of May).
hi i to have pbc, psoriatric arthritis, sjorgens, raynauds, fibromyalgia, all of these autos plus osteo arthr and a weird heartbeat thing my dr z im just auto immuney person painkillers as in paracetamol and urso for the liver and thats it they only really symptomatic relief treat as far as i know infact nothing for the raynauds coz its secondary apart from the liver treatment try not to worry the names and amounts could be dwelled on there depressing on there own take each day as it comes and live as much as poss good luck
Same to you Pattie
You mention high levels of M2 (I guess you mean AMA-M2 ... there are about 9 different sub-types of the autoimmune antibody (AMA), and subtype -M2, is the main one that codes for PBC). However, you do not mention the levels of the liver function blood tests, (lfts) which also need to be known for a formal diagnosis of PBC.
I know - from my own experience - that blood tests, when returned from Labs, always say 'PBC' if AMA-M2 is present at high levels. My results said this and my (new-to-me) GP told me I had PBC - except that I don't! It took a lot of sorting out, and I eventually saw one of the UK's leading PBC consultants, but I had (have!) no symptoms of PBC, and my liver function tests (lfts) have always been normal. It is the results of these tests, the lfts, that you also need to know.
To have a formal/official diagnosis of PBC, you need to have AMA-M2 present in abnormal levels, but you also need abnormally high levels of the specific 'lfts' that are typical of PBC = ALP, ALT, AST, GGT (my consultant sets greatest store by high GGT as well as ALP). AMA-M2 and abnormal lfts, typical of PBC, are the diagnostic criteria that have to be present for a formal/official diagnosis of PBC, so you do need to know the results of all the other blood tests: the lfts. If either are absent (especially AMAs, as some people have PBC but are AMA-M2 clear), but symptoms are present, then a liver biopsy may be needed - it's the 3rd level of diagnosis for PBC.
*[ NB: My new-to-me GP had never come across PBC before (it is pretty rare) and when the Lab results said PBC, she just assumed I had PBC. Someone needs to get the diagnostic labs to make it clear that this is only an indicative test result, not a diagnosis! ]
It may be that your wife does have lfts that indicate PBC - in which case I've wasted your time - but as you don't mention them, I thought it best to warn you. If it is just the AMAs, your wife will need lft tests, annually, in case PBC develops, but will not need urso medication unless PBC develops; also, AMAs alone should not affect insurance (eg travel).
I hope this helps, but apologies if it's not applicable. You may know that the lft levels are also typical of PBC, but just didn't mention it . The meeting with the liver consultant should sort everything out, and I hope all goes well for your wife, but meanwhile, check out all the info on the 'PBC foundation' website. They are the UK-based PBC support Group, and they set up this forum on 'Health Unlocked' - there is a link to their website at the top of this page.
Take care, Gritty.
Gritty, thanks for the detailed response. I’m not certain about the lft results. However, (and if I omitted this information I apologize) the pathology report from the liver biopsy done 2 weeks ago shows bile duct destruction as “being evident” and portal fibrosis in the bile ducts. I’m looking forward to getting complete information from the hepatologist in a few weeks. A liver biopsy done in 2010 showed no bile duct destruction so the diagnosis wasn’t limited to AMA levels; it was merely the basis for suspecting PBC that unfortunately was confirmed with the most recent liver biopsy results. We’re hoping that you’re right but realistic about the challenges ahead facing us.
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