An interesting twist...

[DStaelens]

A little more back story on the recent diagnosis, due to COVID, NYS lockdowns, etc., the ability to actually perform a biopsy of any sort was delayed until 2 weeks ago. Due to the high concern over what looked like rapidly deteriorating kidney conditions, all the symptoms that fit the GPA bill, and ANCA tests that showed they were extremely high, it was decided to forge ahead with the Rituxan infusions. I and the doctors agreed that was the best course of action given what was known at the time and the limitations that were imposed.

Now... The biopsy results come in and the kidney specialists tell me they are completely surprised by the results and don't see ANCA related vasculitis, but instead IgA vasculitis in the kidneys (along with some other things like thin filters, etc.) and now think it could be Henoch-Schönlein purpura. I'm still trying to process if this is good, bad, indifferent news. Right now the plan is to taper off the steroids and basically see what happens. The similarities between the two is quite high, and a lot of the timing and times of year HSP is known for fit. But he almost alluded to a possibility that someone could have both? Any of you folks run into something similar or have knowledge of it?

I have a face to face meeting next week with the RA to go over everything, if nothing else, it's a plot twist in the story.

Hope everyone is doing well!
Thanks,
Dave

[richard052018]

Interesting diagnosis on the Kidney biopsy. Was your PR3 also positive (PR3 is part of the ANCA panel for both Labcorp and Quest) in addition to your ANCA (assuming ANCA-C as opposed to ANCA-P)?

I did some digging on Henoch-Schönlein purpura.

From UNC Kidney Center:

"IgA vasculitis has no known cause. It occurs most often in the spring. It frequently follows an infection of the throat or the respiratory tract. It seems to represent an unusual reaction of the body’s immune system. This is in response to this bacterial or viral infection. Aside from infection, certain medications can also trigger the condition."

https://unckidneycenter.org/kidneyhealthlibrary/glomerular-disease/iga-vasculitis-formerly-henoch-schonlein-purpura-or-hsp/

Have you completed all 4 Rituxan infusions?

If you have Henoch-Schönlein purpura that is great news as the prognosis is outstanding and there is a far more limited chance of a relapses.

[DStaelens]

Yes, I have completed all 4, the infusion center was allowed to let me in in June whereas I couldn't get in for the biopsy until this month. PR3 was positive and ANCA-C pattern was positive.

Thanks for that link. I did get a rash on my legs that disappeared quickly, but it was a heavy spread of very dark purple blotches. And oddly, every time I can think of it cropping up, it was after a cold, sore throat, and typically was the worst in fall through spring months. On one occasion the blotches that normally appear on my hands resolved almost by themselves when an irritant from loose packing was removed after a sinus surgery. That's actually what tipped me off that some sort of immune system thing was happening causing me to search out an RA. There are a lot of symptoms we attributed to GPA like voice changes, constant sinusitis, etc. that don't seem to fit. Not sure if 2.5 years of not getting it treated correctly just lead to a more intense version of it?

As an engineer I find this all fascinating.

[andrew]

Wow - plot twist indeed. I've never heard of Henoch-Schönlein purpura. Google tells me it's treated with steroids so at least you've been on the right course of treatment :) I also see that it's sometimes called Spring Fever. Doesn't have the same ring to it as Henoch-Schönlein purpura :biggrin1:

What kind of engineer are you?

[richard052018]

Yes, I have completed all 4, the infusion center was allowed to let me in in June whereas I couldn't get in for the biopsy until this month. PR3 was positive and ANCA-C pattern was positive.

Thanks for that link. I did get a rash on my legs that disappeared quickly, but it was a heavy spread of very dark purple blotches. And oddly, every time I can think of it cropping up, it was after a cold, sore throat, and typically was the worst in fall through spring months. On one occasion the blotches that normally appear on my hands resolved almost by themselves when an irritant from loose packing was removed after a sinus surgery. That's actually what tipped me off that some sort of immune system thing was happening causing me to search out an RA. There are a lot of symptoms we attributed to GPA like voice changes, constant sinusitis, etc. that don't seem to fit. Not sure if 2.5 years of not getting it treated correctly just lead to a more intense version of it?

As an engineer I find this all fascinating.

Gotta be honest bro I'm floored by your diagnosis. I've been told greater than 95% chance of GPA with positive C-ANCA/PR3, so I never questioned my diagnosis. I had a "negative" Kidney biopsy myself, ie, no signs of vasculitis Kidney damage. My vasculitis came on in the spring after a serious tooth infection and associated extraction/bone graft for implant. Now I'm asking myself do I have Henoch-Schönlein purpura too? I responded VERY WELL to Prednisone alone. It would be a real shame if I'm allowing these drugs to destroy my body for no reason.

[DStaelens]

Been a couple different types, I was first a welding engineer, currently a computer engineer, with a few different things in between. ;-)

[DStaelens]

Gotta be honest bro I'm floored by your diagnosis. I've been told greater than 95% chance of GPA with positive C-ANCA/PR3, so I never questioned my diagnosis. I had a "negative" Kidney biopsy myself, ie, no signs of vasculitis Kidney damage. My vasculitis came on in the spring after a serious tooth infection and associated extraction/bone graft for implant. Now I'm asking myself do I have Henoch-Schönlein purpura too? I responded VERY WELL to Prednisone alone. It would be a real shame if I'm allowing these drugs to destroy my body for no reason.
Interesting, I had flair ups after sinus surgery and after very bad tooth infection requiring a root canal. I found some more information here: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5087233/
Specifically (And I don't pretend to fully understand it):
Among the main differential diagnoses of HSP are multiform erythema and granulomatosis with polyangiitis. Even though clinically similar in some cases, HSP originally does not present a profile of neutrophil cytoplasmic antibodies, like in granulomatosis with polyangiitis, or Wegener's granulomatosis.4 (https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5087233/#r4),6 (https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5087233/#r6),7 (https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5087233/#r7),10 (https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5087233/#r10) These antibodies are associated with necrotizing vasculitides and are classified according to its target antigen in two patterns: the specific pattern for myeloperoxidase (MPO-ANCA) of perinuclear presentation (p-ANCA), and the specific standard for proteinase 3 (PR3-ANCA), of cytoplasmic presentation (c-ANCA).1 (https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5087233/#r1),6 (https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5087233/#r6),7 (https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5087233/#r7) Commonly the c-ANCA antibody is an IgG immunoglobulin, but it may be of type IgA or IgM.6 (https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5087233/#r6) The lack of involvement of the respiratory tract and evidence of granulomatous infiltration - characteristic presentations of necrotizing vasculitides - reinforces the diagnosis of HSP with positive c-ANCA.1 (https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5087233/#r1),5 (https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5087233/#r5),6 (https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5087233/#r6)

[annekat]

I have heard of people having more than one autoimmune condition, even as many as 3 or 4. I guess that would mean you could have two forms of vasculitis. I'm not as up on the technical data as some, though I've probably absorbed a lot through osmosis. If it turns out they are ruling out, WG/GPA, perhaps you should stick around in case they change their minds back again. People have done that and been considered honorary members for life, and anyone with another form of vasculitis would have a lot to contribute! Best of luck as they figure it out, and stay safe around Covid, our unwelcome vistior from outer space, it would seem.

[DStaelens]

So an update: Started to feel pretty good around the 8 week mark after the first Rituxan infusion. Feeling almost normal now and am tapering down on the prednisone quite a bit. Went to another specialist to get a third opinion on the diagnoses and what is going on. He confirmed that A) I do have GPA, and B) it hasn't entered my kidneys, I just happen to have another thing going on that luckily isn't so bad it's an issue but is something to keep an eye on.

With the ANCA/PR3 levels as high as they were/are, he also recommended maintenance infusions of Rituxan and continued tapering off the steroids. So not much of a plot twist, but still interesting I think if someone else runs into a similar scenario. The doctor at Capital District Vasculitis in Albany was phenomenal to speak to help gain an understanding of all the things happening. He easily spent an hour with me going into any details I needed.

The Rituxan infusions have been almost magical for me, I feel better than I have in over two years. Actually cleared to go do things I love doing again like diving!

Stay safe, and take care,
Dave

[vdub]

Very interesting twist. You are too new a member to remember "Jack". Jack was from the UK and was one helluva guy. He died from wegs around 2011 or 2012. Anyway, Jack often said, "just because you have one bad disease, doesn't mean you can't have another". And, he was so right. I think many of us have multiple diseases. Depending on how you count, I have somewhere between 3 and 6 -- some are dependent on or because of others, whereas, some are totally unrelated. All are no fun.