A Year of GPA

I formally received a diagnosis of GPA on Sept 16 2024. I’d known for a few days thanks to the miracle of MyChart test results and a lot of web searching. The formal call with my rheumatologist however was Monday Sept 16 2024.

I was going to tell the story of how I got to the diagnosis but I think it may be more important to talk about the time since.

We need to talk…

On Sept 16, my rheumatologist called me to talk about my test results. If you’ve ever had a partner say “we need to talk”, in that one particular tone of voice, then you know how my doctor started the call. My C-ANCA came back as 1:320. Normal is 1:20. My anti-PR3 antibodies came back as 123.2. Normal is 1.0. C-ANCA is about 90% specific for GPA. Couple it with high anti-PR3 antibodies, and you’ve won yourself a prize.

To be honest, I don’t remember a lot of what she said. I process things on a delay and she threw a lot of information at me all at once. But it came down to: this isn’t a death sentence but it is a life sentence. Some folks achieve full remission. Many do not. The disease is super rare but it seems we caught it early. We need to run some more tests. We need to start treatment immediately.

So we ran tests. The next day, I gave up nine vials of blood to see if my immune system could handle treatment. The tests came back ok. Not great. I was borderline low already but still technically normal.

Two days after that, I had a lung function test. Most folks who get a GPA diagnosis present with failing kidneys or seriously impaired lung function due to infiltrates. I passed at 105% expected lung function. (I grew up in choirs and other vocal pursuits. I suspect 105% was a decrease in function over time for me tbh.)

Then I had a week off of work, a pre-scheduled vacation week. Instead of vacation, I got two CT scans and three MRIs. The CTs were for the GPA diagnosis. The MRIs were attempting to get more information on whether all my joint pain was mechanical or nerve.

The CTs found.. well, they found a lot but most of it subclinical. Look, don’t get scans unless you want to find something, particularly in your 40s. The scan will almost always find something weird and the vast majority of the time, you can’t do anything about it. All you can do is write it down and see if it changes in a couple years.

For these purposes, though, the chest CT found granulomas (the G in GPA) in my lungs, old ones, probably from the 2019 attack. Not enough to cause problems but enough to confirm diagnosis. The doctors did not feel a need to try and get one in a biopsy, thankfully.

As for my joints? Well, I’ll put a different page up eventually about what else is wrong with me soon. Sadly, GPA isn’t the only band playing in town right now.

With the tests back, mostly as expected, we started treatment. 20mg of oral prednisone daily to start. The usual plan for GPA is to start with a medium to high dose of glucocorticoids and then swap that for methotrexate or rituximab, depending on disease severity. So we started at 20mg of prednisone a day, and started 15mg of methotrexate a month later.

And then shit got dark…

My body generally hates glucocorticoids and methotrexate. Gastritis, ridiculous IBS, nerve pain, occipital migraines, both constipation and diarrhea. Brain fog and ME/CFS-style fatigue. I lost the use of my left hand for two days. I gained the ability to feel all my teeth individually including my wisdom teeth that had been removed months prior. I lack a uterus but I understand that my cramps likely came close to PMDD levels. (I said “close” but I’m quite sure I didn’t achieve full enlightenment. Y’all have it rough.)

Naturally, I got the traditional moon face and some of the weight gain.

For two months, at 15mg of prednisone and above, I stopped having an internal monologue and processed everything externally and verbally. This is interesting because I’m autistic and tend towards functionally mute. I was sitting at the dinner table one evening and noticed my partner giving me a bizarre look. I asked about it and they said “you haven’t stopped talking for over a half hour. This is literally the most I’ve ever heard you say at once in our twenty years together”.

I need to emphasize here that I was on relatively low doses of everything at this point. 20mg of prednisone is barely “medium” strength dose. Many folks start treatment at 40mg and 60mg of prednisone. I was also at 15mg of methotrexate which is a small starter dose.

And then shit got darker…

The goal with treatment at this point is to beat down the GPA until its in remission, taper off of the steroids, replace them with methotrexate, and be happy in remission land. It was rough, way rougher than anyone expected, but there would be an end to this, in happy remission land. I just had to tough it through.

Hope is dangerous. In fact, it’s been one of the larger dangers for me throughout this last year. I had hope for remission. I had hope to taper off of steroids. I had hope that my various reactions to treatment would cease.

I no longer have hope.

In the first attempt to taper off of steroids, I experienced a flare / relapse that was worse than any symptoms I’d had to date. It is possible, it turns out, for every bone in your body to hurt. It is possible for every piece of connective tissue to hurt. It is possible for those to happen simultaneously. I would like to never experience that again. Except I did, during the second attempt to taper off of steroids.

I stopped trying to taper off of steroids in early 2025. The minimum viable dose for me currently is 5mg of prednisone daily. Any less than that and my bones hurt. Any more than 10mg and gastritis takes over and ruins the party.

And then things normalized…

By early 2025 though, I was in somewhat of a rhythm. Methotrexate day was a wash, don’t make any plans, prepare to hole up in a dark quiet room near the bathroom. The first couple days after, my joints would paradoxically hurt more as the vasculitis inflammation subsided but the non-GPA arthritis took over. My gut was always a mess but it was a bit better in the early days after a methotrexate dose. Usually, three days after a dose was my peak. I’d start to slide down after that and start experiencing symptoms again on the day before a dose.

I was still plagued with brain fog and fatigue, gastritis, chronic nerve pain. But my lungs were fine, kidneys fine, no infections, and my joint point was were really improved.

So it wasn’t fun but life had become somewhat predictable.

And then I got sick…

I got a middle ear infection out of the blue in May and needed antibiotics. For antibiotics to be worth anything, I had to stop the immunosuppressants, specifically the methotrexate, for at least a week, two to be safe. Also, a random ear infection is how my big flare in 2019 started (and the smaller flare in ‘22). I wanted to know if GPA was impacting my sinuses and ears now.

First we had to eliminate normal causes for ear and sinus infections. I ended up on two rounds of antibiotics and I was off of methotrexate for most of a month and a half. The antibiotics cured the ear infection but not the sinus congestion. Then I was off to a CT scan of my sinuses which showed nothing at all. Every thing’s normal in my sinuses. There are no granulomas or other nasties in my sinuses which is great news. That didn’t explain why I have little airflow through my right sinus.

In August, my head/neck doctor thought we should give nasal glucocorticoids a try. Nasal steroids shouldn’t be too bad, they said. Don’t have to worry about the side effects from regular oral glucocorticoids they said.

Friends, they were wrong. The gastritis and related gut issues started at the three day mark. The occipital migraines started a day later. The brain fog, the nerve pain, all of it came roaring back like I’d added an additional 10mg of prednisone to my life. It didn’t even occur to me that the nose spray might be the issue until I went to see my primary care.

I was on nasal spray for about three weeks. And here we are, about three weeks later, and my gut is just now returning to baseline. I learned a couple of lessons here. One, no more glucocorticoids for me unless absolutely necessary (and I need to figure out a food solution for when they are). Second, no more going off of methotrexate unless it’s absolutely necessary. My GPA is waiting in the wings, right now, just biding its time for me to slip up. Which is why…

And then I changed to subcutaneous methotrexate…

In August, my rheumotologist suggested I switch from oral to subcutaneous methotrexate. It had become clear that I wasn’t responding to treatment like most folks do. I shouldn’t be relapsing and rebounding like this and jumping my steroid dose was clearly not an easy option. What if I wasn’t absorbing the drug properly because my gut’s a mess? What if the methotrexate is part of the gut problem too and we can take it out of the equation? After all, many patient experience a drop in adverse gastric events by changing to subq methotrexate (at the cost of some additional brain fog and fatigue) [1].

With some great trepidation, I agreed. I struggled with this change, to be honest. I still do. Injecting myself weekly is a stressful event and my generalized anxiety is upset by it.

I want to tell you that miracles occurred and everything is magically better. I want to tell you that no darker tales await. Sadly, I can’t. But there is a bright part.

First, the horrors. It turns out that I am not “most patients”. My day-of gastric adverse events are so much worse. Mind-bending cramps, diarrhea, occipital neuralgia, brain fog (though that might be from the neuralgia). About an hour after I inject myself, the party gets started and it usually doesn’t let up until the next day. It’s real bad, not universally, just about half the time. Of course, the unpredictability is its own problem.

But there is a bright side. On the other days in the week, my non-mechanical joint pain is nearly gone. Fatigue and brain fog are so much less. I have my brain back (mostly) and feel better in that regard than I have in a year, maybe two or more. My mood’s generally better and I’m getting back into my hobbies.

If it weren’t for the bright side, I’d go back to oral methotrexate in a heart beat. These day-of side effects are pretty terrible. But the bright side exists and is great and worth, so far, the horrors.

And here we are…

I have second thoughts about publishing this post, if I’m honest. This is probably the darkest thing I’ve posted publically since an accidental permissions oops on livejournal back in the 00s. It was pretty awful for me to relive typing it out and I’m pretty sure you didn’t have any fun getting here.

But I put my site back online with the intention of documenting my saga on the hopes that it helps someone or helps them feel less alone, to share the story of my limited-subset GPA. So maybe it’s important to say to you that this disease may not be actively trying to kill my major organs but it fucking sucks nonetheless. Maybe it’s important to share publically the stuff my doctor isn’t concerned about because the meds are required and there’s nothing we can do about the side effects anyway. Maybe its important to record that these meds are awful and there’s nothing I can do but soldier onwards.

I don’t know. But I will say that I’m allowing myself a little hope that subq methotrexate will make my life a lot better for five to six days a week. And that’s not nothing. I like having my brain back, to have hobbies, to feel competent in my job, to move without so much pain, even if it does mean one to two days of mind-destroying gut symptoms and migraines.

And maybe that’s the most important part for me, typing out and sending out publicly that after a year in which I lost all hope for a better future, a year where I asked my doctor to stop giving me anything but the cold hard facts, that after all this, I have a kernel of hope that maybe things will be 5/7 ok. I’ve gone from maybe 1/7 to 5/7 and that’s worth celebrating and worth a little hope.

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