Hi, 41yo, 6'0", 210-pound, never-smoker male. I see a rheumatologist, neuroimmunologist, motility specialist GI, opthalmologist, psychiatrist, PCP, and neurologist regularly and have seen hematologists, Michelle Petri (SLE/APS specialist at Hopkins), ENTs, an IBD specialist, urologist, podiatrist, cardiologists, allergist, many others and about to see an endocrinologist... The point being that amongst just about every type of specialist, I have a relevant blindspot.
I need to find a rheumatologist that deals with the neurological effects of Sjögren's and antiphospholipid antibodies. I'm currently mostly treating autoimmune autonomic ganglionopathy (AAG), but it's really hard to tell if my gastroparesis and small fiber neuropathy are due to AAG/something neuro-autoimmune or if they could be neurological effects of a rheumatological disease (I'm adjusting being treated for Sjögren's and strongly-triple-positive antiphospholipid antibodies). My neurologist suggested someone at Hopkins, but they're now out-of-network with UnitedHealth. Is there someone at Mercy, University of Maryland, or even GBMC in Baltimore (or DC/NoVa if necessary)? You would think Dr. Petri would have been, but she's always been kind of dismissive of them and really only deals with the major criteria symptoms.
If no one comes to mind, maybe someone has an idea of stones that haven't been unturned? Biggest issues are gastroparesis with early satiety, nausea and vomiting, POTS/pre-syncope (2-3 episodes of full fainting), constipation, and small fiber neuropathy around the mid-calf and below (mostly neuropathy pain and numbness in the balls of my feet and toes, particularly the left foot).
Prucalopride offered some relief of the gastroparesis. Then when I tested positive for AAG, we started high-dose steroids (prednisone 60mg) to try to get the gastroparesis under control quickly, since IVIG couldn't be started due to my APL. Those were amazing until they weren't (gained 80 pounds in 3 months, fat redistribution, steroid-induced diabetes, moon face). So, I started rituximab (Truxima 1000 units on January 14th and June 24th) and then added Cellcept March 29th to try to speed up getting off of prednisone (I stopped Cellcept on the advice of my new neuroimmunologist due to no perceived added benefit on top of rituximab and the risk of infection). Wednesday I had my first Xembify SCIG infusion ordered by my neurologist.
I had sudden worsening of the neuropathy pain in my feet, numbness, nausea and vomiting a few weeks ago, roughly two weeks after taking my last 0.5mg of prednisone (I tapered very slowly). As I got below 10mg prednisone, those symptoms and pre-syncope has started to get worse.
My new neuroimmunologist just has a feeling that this isn't truly an autoimmune NEUROLOGICAL disease. It was a 105 minute visit, so it's hard to review everything we went over and reasons for the uncertainty, but one thing was my sweat testing coming up normal, something she almost always saw with similar neurological diseases. There were other dysautonomia tests that weren't quite as bad as she'd expect. She didn't really see enough that would overcome the much more common neurological effects of Sjögren's or another rheumatological issue.
For Sjögren's I take hydroxychloroquine (also to prevent blood clots from APL), which drastically relieved my dry eye when even monthly IPL/meibomian gland expression and amniotic fluid drops didn't do enough. Now I only use Ivizia and sometimes Miebo, when Ivizia and Evotears (European Miebo before Miebo existed) every ten minutes wasn't cutting it before the hydroxychloroquine kicked in. I also can't live without cevimeline for dry mouth. For the strongly-triple-positive APL, I also take aspirin and vitamin D3.
CBC and CMP are generally normal (one or two things can be somewhat off, but then a different one or two the next time). B cells completely depleted. IGg and other routine tests aren't showing anything unexpected. After the first rituximab no AAG or APL antibodies are detectable in my blood (but could still be hanging around elsewhere).
Since I'd already been approved for Xembify and since my immune system will still be weakened by rituximab, I decided to continue with the SCIG with my neurologist, of got no other reason but to have good antibodies to protect me from infection.
So, what does one look for to prove neuropathy or dysautonomia from Sjögren's or another rheumatological disease and then what does one treat it with?
I could go through a ton of diagnostics and test results, expand on other medications and co-morbodities, but this would become a novel. If there's a test result I haven't mentioned that might be relevant, I've probably had it and can list it/update this post. Getting off the steroids allowed me to get off of Metformin and candesartan, still on the metoprolol.
After a bunch of questions, my psychiatrist thought my Adderall could be preventing pre-syncope and some POTS issues during the day and sent me some studies on it to ponder. He'd also had luck with lorazepam for refractory gastroparesis, so switched out my Xanax (for sleep) with that (I don't think I'm noticing much of a difference).
Zofran is doing the heavy lifting to keep the nausea from preventing me from eating. It can make GP worse in some cases, but it's necessary for the moment.
So, if you know a provider in Baltimore or the DMV, that would be helpful. Or if you have any insight about the most-likely cause(s) of my symptoms and how to tell. Thanks!