Prognosis, Diagnosis, Options....can anyone provide some guidance?

[eekatz56]

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

[fntn]

I have a disease similar to your wife's PBC, I have PSC.  Both tend to be long term chronic diseases and will have associated AI diseases as your wife has.

She should try to find a hepatologist to get the best advice and treatment programs.  Also the long term cholastatic diseases will cause osteodystropy, so bone density and osteopenia/osteoporosis should be watched.

Hope she remains healthy for many more years.  The progress typically takes more than a decade or so to become end-stage, where a liver transplant would then be needed. I am at end-stage myself. The symptoms will become very pronounced but the course varies for each patient.

Take care.

[eekatz56]

Thank you for the information and your well wishes.  I can't tell which stage she's in.  We've got an appointment with a hepatologist in 2 weeks.  I think since we suspected she had PBC in 2010 when she was diagnosed with Sjogrens Syndrome.  It's now been 8 years since that liver biopsy and with her current enzyme levels, when added to minor itching but constant fatigue, seem to indicate she's no longer asymptomatic (although no jaundice has appeared yet).  I'm not sure what that means and I'm not trying to be pessimistic..... just realistic about the prognosis.  I hope you're in a position to receive and successfully receive a liver transplant soon.