3.9. my GPA is 3.9, drug down by a poor showing in phys ed.
Awful attempt at humor complete.
Look, in the chronic illness game, you take the humor where you can.
My situation is … complex and I’ll talk about the rest eventually. But GPA is the big one, the one that brings all the doctors to the party (well, like two, it’s a small party). What is this thing, and what is my version of it?
Granulomatosis with polyangiitis (GPA) is a type of vasculitis — chronic inflammation in your blood vessels. GPA involves:
- Inflammation in many different types of blood vessels throughout your body (polyangiitis)
- Inflammatory masses called granulomas forming in your blood vessels and organs (granulomatosis)
GPA is necrotizing granulomatous inflammation usually involving the upper and lower respiratory tract, and necrotizing vasculitis affecting predominantly small to medium vessels
What’s this thing do? It restricts blood flow to places like the kidneys, lungs, sinuses and leaves little rocks all over the place. The human body is not normally full of little rocks, if you weren’t aware. It can affect any part of the body at any time in any combination.
The granulomas, the little rocks?, they do… we don’t really know. They impede blood flow, increase inflammation, and the like. They can become pseudotumors, weird bumps on your elbows, or just chill. But normal bodies aren’t full of little rocks and we just don’t know what all happens yet.
How rare is it?
As of 2022, the worldwide prevalence of GPA (how many are actively afflicted) is estimated at 96.8 per million. Worldwide incidence of GPA (how many are newly diagnosed) is estimated at 9.0 per million/person-years. (If we watched a million people for one year, we expect to see nine cases.) [3]
That puts us at around 33k afflicted in the USA and 784k afflicted worldwide. Not quite enough to warrant a dedicated Etsy or Tiktok presence.
I’m told that GPA comes up in med school in a very brief section about vasculitis. It is mentioned because the granulomas are interesting, and then everyone moves on because it’s so rare “we’ll never see it”.
But really, we’re still learning just how rare (or not) diseases like GPA, eGPA and MPA truly are because the relevant antibodies weren’t described until 1982 and the blood test to see them wasn’t widely available until the end of the 90s. So even though GPA was formally described by the Nazis back in ‘36, we’re still developing the data sets.
How bad is it?
Left untreated, average life expectancy is five months, with less than 50% surviving a year. With treatment, 80% survive at least eight years. [1], [4]
As I said earlier, our data is still a little spotty. Until pretty recently, most GPA diagnoses were made on the death bed. Kidneys failing, lung infiltrates compromising breathing, a plethora of mysterious confusing symptoms, and some doctor decides to throw a test on the pile for ANCA and anti-PR3 antibodies and bam we have a diagnosis. Folks with lesser symptoms slipped through the cracks because they didn’t warrant the tests and differential diagnosis. Usually they landed with a diagnosis of something like rheumatoid arthritis which happens to have a similar treatment plan.
Further, GPA tends to present in 45 - 65 year olds, with most being in their 60s due to the aforementioned problems [4]. So “most survive eight or nine years”, in many cases, translates to “and they lived happily (?) ever after.”
No really, how bad is it?
GPA can take a bite out of you in any number of un-fun ways [4], [5], most of which aren’t fatal.
- Fever, weight loss, general malaise (feeling like shit all the time for no apparent reason)
- Recurring, slow resolving ear infections that can result in loss of hearing or deafness
- Inflammation and pseudotumors in the eyes, which can lead to vision loss or blindness
- Frequent nosebleeds, erosion of the nasal septum, collapse of the nasal bridge
- Narrowing the windpipe, aka glottal stenosis
- Lung infiltrates, leading to loss of lung function and bleeding
- Arthritis in… well, everything
- Joint and muscle pain (this is really underselling it)
- Neuropathy, usually bilateral (numbness, tingling, shooting pains in the extremities)
- Kidney failure
Some folks get few symptoms. Some folks get all symptoms. Some folks get new ones like significant gastrointestinal involvement (it me) [6], [7], [8].
But it gets better with treatment, right?
I guess? Yes, it does. But here’s the thing. This is a spectrum. Some folks achieve full remission. Many folks do not. Even the folks who achieve full remission will almost certainly relapse within the first few years.
Most relapses of GPA or MPA occur in the first 12–18 months after cessation of immunosuppressive treatment, although they can appear after more than 10 years after the initial presentation. [9]
In a 2013 study of 838 systemic vasculitis patients, 27% developed new severe manifestations after diagnosis and 37% developed new non-severe manifestations [10].
And even once you get past all that and into remission, you are still probably on an immunosuppressant of some variety. These drugs have their own side effects and their own risks, namely that of secondary infections. (If you stopped wearing a mask, why on earth would you do that? Go buy more.)
To depressingly quote Nefrología [9]:
The improvement of immunosuppressive treatment has transformed vasculitis into a chronic disease and, consequently, the priorities of patients have been readjusted to this reality. Instead of focusing on the consequences of organic damage, patients consider fatigue and chronic pain as the main factors of the disease that impair their quality of life.
Despite the advances of new induction drugs in vasculitis, the benefits in quality of life are modest and rarely become normal.
But you’re alive.
Yes, when treated, the vast majority of us live a long ass time. Is it happy party time? No. If you’re a long term methotrexate user, will you see the sun again? Probably not. If you have to stay on prednisone long term, will you weigh more than you want? Absolutely.
But you will be alive. I will be alive.
This is a life-changing disease, for sure, but the numbers say it is typically a survivable one. Just…. just don’t your hopes up for perfect long-term symptom-free remission.
How bad is it for you, individually?
I have what the Americans call “limited subset” GPA where my kidneys do not seem to be involved. They seem fine, thankfully. (That may not last forever.) Folks like me typically get a diagnosis of fibromyalgia or rheumotoid arthritis, until shit gets dark. In fact, those were the diagnoses I expected. But my rheumotologist threw an ANCA and anti-PR3 test into the mix because why not and here we are.
Symptom wise, here’s where I am today:
- Chronic rhinitis
- Signficiant joint and muscle pain
- Significant gastrointestinal involvement
- Neuropathy
- An abdominal aneurysm
- Might be getting glaucoma from the treatment
I’ve had two major flares of more traditional GPA in the past, in 2019 and 2022. I probably should have gone to the hospital both times but I had crap health insurance and rode it out into an “asthma” diagnosis from a lesser doctor. I had unstoppable ear and sinus infections and coughed my fool head off constantly for a couple months, barely able to breathe, until that “asthma” diagnosis resulted in a treatment of corticosteroids. Fun thing is, the prednisone they give you to squash an asthma breakthrough is the same prednisone they give you to bring GPA under control. The maintenance inhaler they give you for asthma is oral inhaled corticosteroids. So in both cases, the asthma drugs put my disease went back into remission temporarily.
I got diagnosed because the lesser-known symptoms cropped up in 2023 and started getting worse. Joint and muscle pain were/are the worst of them and exacerbated my existing mechanical disability. The vasculitis made my existing arthritis and neuropathy issues worse. My rheumatologist ran all the tests they could. In 2024, ANCA and anti-PR3 tests gave us the initial diagnosis, CT scans confirmed granulomas in my lungs. (A brand new nurse in 2019 spotted the granulomas in my x-rays when making the referral to my first pulmonologist but didn’t know the proper term apparently so the pulmonologist dismissed his concerns.)
Good news is:
- no apparent kidney or liver involvement
- 105% lung function
The bad news is that after a year of treatment, I am not in remission yet. I thought I’d gotten there in March but then I got an ear infection and kind of all hell broke loose. We’re sorting through it and what it means for the treatment plan now.
How are they treating it?
My current treatment plan is 25mg/week of methotrexate (switching from oral to subcutaneous) and 5mg/day of oral prednisone. It’s possible I’ll switch to rituximab infusions in the spring but we’re trying to avoid it, for so many reasons. I don’t know right now if I can avoid it. It’s not been a good symptom month.
How did this happen?
We’re still figuring that out, generally. GPA is an autoimmune disease, in that the body produces antibodies against itself, specifically ANCA anti-PR3 which targets neutrophils, a critical component in the innate immune system [4]. How that gets started, though, is still being researched. There is good progress towards finding genetic markers [11] and growing evidence of environmental and infectious triggers (particularly Staphylococcus aureus) [12].
Why me? There’s no good answer to that because we don’t have a precise etiology of the disease. But it’s clear from the data so far that I did not do this to myself through stress and lifestyle choices, as some might say for autoimmune diseases in general. (Those people are assholes, to be clear.) I lost a dice roll along the way.
What now?
I keep pushing forward. I don’t know what else to do. I’ll do a full post on my head space and my day to day experience at some point. But in the end, I keep pushing forward. I’m holding down a regular tech job, which takes most of my energy in the day. Trying to stay alive (or sometimes just stay awake) after work. I’m doing ok at that so far. Not perfect, of course, but ok.
I often say “hope is dangerous” and hope is not on offer anymore. Even my doctors have learned that lesson. The future does not look amazing, characterized by chronic pain, fatigue, brain fog. But the future does currently look like it exists. Alive is best and there’s little doubt, today, that I’m going to live a good while longer.