Primary Biliary Cirrhosis.

Dear patient,

Thanks for the query. Your case is a typical natural history of autoimmune disorder, mild fatigue, female gender, mild symptoms overall. Now to make the things simple you still require few investigation like ESR CRP, urine routine and microscopy, serum C3 C4 levels, definitive diagnosis with liver biopsy. You should get line immune assay done for ANA to rule out any overlap syndrome. This test will give idea regarding subtype of ANA whether you have Sjogren, MCTD, Scleroderma, Lupus etc.

Why all this? to ascertain what is the diagnosis. Is it just PBC / overlap syndrome etc.

Does high ANA mean a sever disease ? no
Does high AMA is pathogonomic of PBC? no

Do both ANA and AMA can be unified? yes
How do I know that what is going to happen? Patience as we may overtreat you due to high value.

What will Immunologist/gastroenterologist do? He would ask the investigation listed and start you mild on immunosuppressant and review periodically.

How often do i require ANA and AMA to be repeated? not required

Do future low ANA and AMA indicate low disease? no

I hope this has helped. Feel free to follow up.

Thankyou

Thank you for your detailed response. I have had some urine work done already, and could provide some readings to it, but I didn't have significant protein in the urine, but that has varied on different tests. I'm sure you are referring to something more than what I have already had, though. What do you mean overtreat due to high value? High ANA or AMA? I assume I would begin Ursidol for the PBC, and then if overlap whatever treatment for that (meaning your comment on mild immunosuppressant therapy)?

I have an appointment with a gastroenterologist on March 18, since in my area liver specialists only are fairly rare. Do you suggest I see any other specialist?

Hello,

If we approach your concerns in a different way - How many percentage of patients with ANA and AMA at your age and sex and family history of PBC actually develop PBC and in how many years. - as your LFTs are normal. So a diagnosis of PBC just based on ANA or AMA values is not recommended (and has a risk that you may be over treated - especially for any possible overlap ). The specific treatment of overlap depends on what kind of overlap you have. This will involve few tests, might involve some waiting and further followup.

Thank you

Just one more question. My Igm was negative. I have read that almost everyone with PBC has a positive IGM. Is this something that would or could develop later? Also the SMA was negative. You also mentioned my LFT's were normal. My ALP is 500, not normal and my alt and ast are elevated (not over 2 x's though) Would IGM being negative mean anything? I know I will probably need a biopsy. I have an appointment with a specialist in a couple of weeks.

Dear Maam,
I have a few observations, Your GGT and ALP are elevated. That is why I felt you need to get liver biopsy done. AMA and ANA are are strongly positive. If the liver biopsy is suggestive of inflammation but not suggestive of PBC. And mild SGOT/SGPT i.e ALT/AST elevation. It is significant and is suggestive in your context. I am not worried about IgG versus IgM at this juncture. I would like to see the results of your liver biopsy to further comment.

Regards.

Hello! You mentioned you would like to see my liver biopsy results. I have those now. I am going to see my doctor tomorrow, but I would like another opinion before I see him. My biopsy says I have PBC with possible AIH overlap, though they can't determine that at this time without further testing. What I am confused about is the stage of my PBC. From what I am reading I think I know what the stage is, but not sure. the microscopic description is a follows.

H&E stained sections of the liver needle biopsy show preservation of the normal hepatic architecture. The needle core biopsies include parts approximately 31 portal tracts. The portal tracts show mild to moderate infiltration by lymphocytes along with a few plasma cells, neutrophils, and rare eosinophils. There are epitheloid granulomas in many of the portal tracts with some of the epithelioid granulomas occurring surround interlobular bile ducts. Interlobular bile ducts appear to absent in several of the portal tracts. Most of the portal tracts show mild interface hepatitis. There are scattered foci of lobular lymphocytic infilitration. Some of the lymphoctic infiltrates are associated with epitheliod hisiocytes or Kupffer cell proliferation. There are a few lobular epithelioid granulomas. There is no significant heptocelluar steatosis. No PAS positive diastase resistant intracytoplasmic globules are identified in PAS with diastase stained sections. There are rare sinusoidal PAS positive diastase resistant macrophages or Kupffer cells and a few PAS positive diastase resistant macrophages in several of the portal tracts. No hemosiderin is identified in hepatocytes of in Kupffer cells in section stained for iron. Reticulin stains show a normal pattern with plates of hepatocyts predominately a single cell in thickness. Trichrome stains show a focal portal and periportal fibrosis. No bridging fibrosis or cirrhosis is identified. Cytokeratin 7 stains show loss of interlobular or bile ducts in several portal tracts. Cytokeratin 7 stains highlight mild ductular proliferation in a few portal tracts. Cytokeratin 7 stains also show cytoplasmic and membranous staining of periportal hepatocytes around some but not all of the portal tracts consistent with chronic cholestasis. PAS with diastase, iron, trichrome, reticulin, and cytokeratin 7 controls stain appropriately.

I see my doctor at 1030 am EDT tomorrow, so if I could get an answer today that would be great.

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