Trying to figure it all out.

[robertlevigarrett]

Greetings!

39 year old male here. Diagnosed with Autoimmune Hepatitis about two years ago. Was on prednisone and azathioprine last year to get things under control. Stopped prednisone earlier this year, continuing the azathioprine (175mg) for disease maintenance.
Shortly after stopping the prednisone, I had some new symptoms emerging and started back down the “what the hell’s wrong with me?” road.

At first my docs were thinking RA (joint pain, fatigue, elevated RF, etc.). After further testing (clean x-rays, RF back to normal), my rheumy was unconvinced that it was RA and sent me to a neurologist. By this time, I was was having mild dizzy spells/balance issues, having some numbness/tinging in my hands and feet, burning sensations in my left leg and left side of neck, minor cognitive issues, spasticity, random pain all over my body, ED, chest discomfort and weird occasional shortness of breath.

Brain MRI showed a few small lesions “consistent with a demyelinating disease like MS”. My neuro ordered lumbar puncture and spinal MRIs along with about 30 different blood tests. CSF showed elevated proteins indicating inflammation but no other markers for MS. To my knowledge, there were no lesions detected on my spinal MRIs (I see neuro in a couple days).

One of the blood tests was an ANCA panel, which showed elevated PR3 (4.6) and an atypical pANCA titer >1:640. C-ANCA was "indeterminate" (P-ANCA and MPO were both negative). At this point, my neuro sends me back to my rheumy, but turns out he's out on medical leave until June, so I get a new referral to a new rheumy who, because of the positive ANCA, gets me in the next day. (I was also born with only one functioning kidney).

Based on my presentation, my rheumy put me back on a prednisone taper and started me on 400 mg of Plaquenil. He tells me the high atypical pANCA titer is likely from my autoimmune liver disease, and went ahead and ordered another ANCA panel as well as some kidney function tests.

Results of kidney function test came back normal. PR3 is now 4.9 and my C-ANCA is positive at 1:160. I know these numbers aren't crazy high or anything.

Fortunately, I've been taking a managing dose of azathioprine for almost two years, so I've been keeping whatever is going on "at bay" for the time being. Not to mention the 10 or so months of prednisone last year. At the same time though, I feel like my presentation might be a little atypical, because I'm already partially treating whatever is going on.

I'm waiting to hear back from my rheumy to get his thoughts on the results, but I stumbled upon this forum in my research and thought I'd share my story up to this point. Maybe get some of your opinions on the matter. I keep reading that a positive C-ANCA and PR3 are highly specific to Wegener's. I've also read that Wegener's can have some central nervous system involvement, which might explain the elevated proteins detected in lumbar puncture as well as the neurology related symptoms.

Any thoughts based on your experiences? Any advice on handling my follow-up appointments? Questions to ask, things to remember, etc.

I love that this forum exists! Thanks for taking the time to read!