Author Topic: Anyone have small vessel Vasculitis with their Sjögren’s?  (Read 2068 times)

MAT51

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I am looking into whether I might have a type of Vasculitis secondary to my Sjögren’s. In fact I’m starting to wonder if I may have IgA Vasculitis/ Henoch Schönlein Purpura - which is more common in children apparently - but can occasionally be found in adults too.

I have had +1 to +3 trace blood in my pee for years now. My bladder was checked out by cystoscope and all was very healthy. The mycroscopic haematuria disappeared while I was on the highest dose of Cellcept and my inflammation markers lowered to mildly raised. But I was taken off Cellcept in February with a “wait and see” approach being adopted by my rheumatologist and her vascular colleague. They didn’t feel there were clear enough benefits and have told me that my main problem is small fibre neuropathy - which they can’t treat apart from for pain with the epilepsy drugs. 

However, over the past couple of months I’ve suffered awful leg pain along with bowel and bladder issues - assumed to be Cystitis due to blood and leucocytes in my pee and a bacterial vaginosis ecoli infection. But finally, 3 courses of antibiotics later, the hospital lab can find no sign of infection in the trace blood. So my GP says it’s all unrelated. She and my physiotherapist agree that recently identified herniated discs don’t account for my numbness, tingle and pain in arms and legs, although they are probably adding to the overal misery.

Plus I’ve developed a resting tremor now which worsens when I’m in pain or unwell or extra fatigued. The neuro PT implies that this could be Parkinson’s but I don’t think it is. She is really helping me with the proprioception issues caused by the SFN everywhere but admits she knows little about Sjögren’s as she mainly works with MS patients.

But I must say that the coinciding blood in pee and related symptoms worry me - particularly as i am having severe pain in my groin and genitals often - which they suspect is the small fibre neuropathy advancing. I’m now trying Lyrica with some misgivings - but I do need something for the pain. I’m only on week 2 so taking 100mg - rising slowly to 200mg or more depending on my tolerance.

So about 3 weeks ago I noticed this splatter of strange spots on my feet and ankles. They weren’t itchy or raised, were non blanching and flat - but not that many - I counted about 19 in total. Also some fainter small ones on my shins.  At same  time I was having sweats and nausea with bouts of diarrhea. But I decided that the spots and other unwellness were maybe just a delayed reaction to all the antibiotics so ignored them. Then they stayed for a few weeks getting darker and stranger before finally fading away now - not gone entirely yet but barely noticeable enough to show.

I’ve had these spots before coming and going on my shins for years when I flare up but not as large or dark and pronounced as these guys. I did see another GP a week ago as I was feeling so rough with the pelvic and back pain and I wanted to know if I have kidney problems. She looked at my recent bloods and scans and said not evident so far but examined the petechiae on my feet and agreed with me that some kind of small vessel Vasculitis was the likely explanation. She told me to photograph them and show to the dermatologist and rheumatologist - both of whom I see next week. She told me to emphasise that these are non blanching petechiae rather than a rash. I duly photographed them so have a record at least. I’ve read that diagnosis requires biopsy of the spots within 48 hours of their appearance. Also that I may need biopsy of my kidneys. 

My IgA in my blood is always slightly elevated above range so this could fit. But IgA Vasculitis is very rare and I’m just wondering now how many of my symptoms and signs might actually have been Vasculitis related all along rather than all Sjögren’s? And whether my rheum will agree to get me back on Cellcept at last - or try something else - although I’ve so far tried most of them over the past 7 years.
« Last Edit: August 13, 2018, 12:53:38 AM by MAT51 »
Hashimoto's, seronegative RA, Primary Sjogren's, small fibre nld polyneuropathy, hypertension, IBS-C, GORD, BMS, highly allergic disposition!

Tiff123

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Re: Anyone have small vessel Vasculitis with their Sjögren’s?
« Reply #1 on: August 13, 2018, 03:43:20 PM »
New here, but this thread is up my alley big time.

Been diagnosed with Sjogren's several times via testing. My antibodies for it are through the roof! I have dry-ish skin all the time and dry-ish eyes but I don't have the typical brutal suffering for it (at least yet?).

What led me to get testing was developing serious purpura rashes all over my legs two years ago. Huge patches of that crap that would come and go. Often they were monthly on the dot, right around my period. Most doctors screamed vasculitis but even my skin biopsy would not conform typical vasculitis. :o Most thought I was allergic to something.

I had tried to eliminate various medications, nothing had worked. Tried gluten free, nope made it even worse. The food thing never seemed to help.

I had gone into remission for months but still would get little red bumps all over my shins and ankles off and on.

It came back with rashes on my legs and thighs (in patches) again in February and sometimes around my belly button and lower stomach and even on my left arm.

Had a few one week courses of prednisone too, it helps when I am on it but when I go off I am back to square one. My doctor won't let me go on it very often anyway.


Unlike you, I never had blood in urine. Urine was always tested fine. Kidneys fine, all internal organs fine. Have had a million blood tests. Elevated ESR rates are usually all I get that go up and down in waves.


Just diagnosed with sjorgren's and continuous mini blood rashes and occasionally swelling and bruising in feet and ankles.

Only thing I ever found to help work to an extent and lessen symptoms and even put me into remission once in two years was systemic enzymes. So I just went back on them two days ago hoping it would help again.


Just very odd. I can't figure out the correlation between sjogrens and these petechiae/pupura rashes that come and go and have been my #1 symptom. Nothing in blood tests have ever come up that screamed to any doctor "serious!". My skin is very fair so sadly any of these blood spots stick out big time. I wear pants all year round.

Tiff123

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Re: Anyone have small vessel Vasculitis with their Sjögren’s?
« Reply #2 on: August 13, 2018, 03:44:17 PM »
Sometimes my rashes have been itchy, if it's a good sized one they burned like heck. I often had a steroid cream to use. The smaller patches and random spots don't usually itch.

jazzlover

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Re: Anyone have small vessel Vasculitis with their Sjögren’s?
« Reply #3 on: August 13, 2018, 06:16:44 PM »
Look into possible EDS or the vascular form of it. .. it has co-morbidity with Mast Cell Activation Syndrome or Mastocytosis.
Mast Cell Activation Syndrome (MCAS), Salicylate Sensitivity,  Interstitial Cystitis,  gluten intolerance, Raynaud's, Sjogren's, A-fib; cytomegalovirus, recovered from Lyme disease

anita

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Re: Anyone have small vessel Vasculitis with their Sjögren’s?
« Reply #4 on: August 13, 2018, 07:09:07 PM »
I have had blood in urine for 20 years...they have never found the cause, and just assume it is inflammatory from Sjogren's.  I do have livedo Reticularis, and this is from spasms of the medium sized blood vessels causing goofy/lacey rash type look on the skin.  It gets worse with cold.  I have it from head to toes, including torso, but most have it only on legs.  It can be related with some types of vasculitis.  I can't remember, do you have this?

52 yr old SjS, APS w/strokes, Autonomic Neuropathy, PN, Nephrogenic DI, (CVID) IgG def., Cushing's, Asthma, Gastroparesis.  Sero-neg w/+ lip biopsy.  Meds: IVIG & pre-meds, Arixtra, Aspirin, Plaquenil, Cardizem, Toprol XL, Domperidone, Nexium, Midodrine, Symbicort, Fentanyl, Percocet, Zofran

MAT51

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Re: Anyone have small vessel Vasculitis with their Sjögren’s?
« Reply #5 on: August 13, 2018, 09:58:37 PM »
Look into possible EDS or the vascular form of it. .. it has co-morbidity with Mast Cell Activation Syndrome or Mastocytosis.

Thanks Jazzlover. I hope it’s IgA Vasculitis because, although this can affect the kidneys - the prognosis is better. But that said - my Neuro PT said I’m hypermobile and I have many signs and a family history suggesting EDS. There’s no likelihood of my getting a diagnosis where I live and I can’t afford to pay to get one in London.
Hashimoto's, seronegative RA, Primary Sjogren's, small fibre nld polyneuropathy, hypertension, IBS-C, GORD, BMS, highly allergic disposition!

MAT51

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Re: Anyone have small vessel Vasculitis with their Sjögren’s?
« Reply #6 on: August 13, 2018, 10:05:38 PM »
New here, but this thread is up my alley big time.

Been diagnosed with Sjogren's several times via testing. My antibodies for it are through the roof! I have dry-ish skin all the time and dry-ish eyes but I don't have the typical brutal suffering for it (at least yet?).

What led me to get testing was developing serious purpura rashes all over my legs two years ago. Huge patches of that crap that would come and go. Often they were monthly on the dot, right around my period. Most doctors screamed vasculitis but even my skin biopsy would not conform typical vasculitis. :o Most thought I was allergic to something.

I had tried to eliminate various medications, nothing had worked. Tried gluten free, nope made it even worse. The food thing never seemed to help.

I had gone into remission for months but still would get little red bumps all over my shins and ankles off and on.

It came back with rashes on my legs and thighs (in patches) again in February and sometimes around my belly button and lower stomach and even on my left arm.

Had a few one week courses of prednisone too, it helps when I am on it but when I go off I am back to square one. My doctor won't let me go on it very often anyway.


Unlike you, I never had blood in urine. Urine was always tested fine. Kidneys fine, all internal organs fine. Have had a million blood tests. Elevated ESR rates are usually all I get that go up and down in waves.


Just diagnosed with sjorgren's and continuous mini blood rashes and occasionally swelling and bruising in feet and ankles.

Only thing I ever found to help work to an extent and lessen symptoms and even put me into remission once in two years was systemic enzymes. So I just went back on them two days ago hoping it would help again.


Just very odd. I can't figure out the correlation between sjogrens and these petechiae/pupura rashes that come and go and have been my #1 symptom. Nothing in blood tests have ever come up that screamed to any doctor "serious!". My skin is very fair so sadly any of these blood spots stick out big time. I wear pants all year round.


Hi and welcome!

My spots sound less significant than yours I must say. And they literally don’t itch or hurt at all - not even slightly. However I do get little spots around my face that also cluster in lines and itch slightly as they form and are non blanching too. I’d say it’s far from my worst symptom though - the painful neuropathy, weakness, tremors and IBS-c/ dysmotility  problems are my worst symptom by far.

Keep posting on the main forum and follow this post for suggestions from others here though.
Hashimoto's, seronegative RA, Primary Sjogren's, small fibre nld polyneuropathy, hypertension, IBS-C, GORD, BMS, highly allergic disposition!

MAT51

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Re: Anyone have small vessel Vasculitis with their Sjögren’s?
« Reply #7 on: August 13, 2018, 10:31:16 PM »
I have had blood in urine for 20 years...they have never found the cause, and just assume it is inflammatory from Sjogren's.  I do have livedo Reticularis, and this is from spasms of the medium sized blood vessels causing goofy/lacey rash type look on the skin.  It gets worse with cold.  I have it from head to toes, including torso, but most have it only on legs.  It can be related with some types of vasculitis.  I can't remember, do you have this?

Hi there Anita. Yes I do have livedo reticularis quite dramatically on my feet when they are exposed to air - even warm-ish air - and my knees too. When I noticed these petechiae my feet were dark grey and the petechiae spots stood out like a pink splash with paler surrounds. Then they became dark purple over the next few weeks. Now they have all but faded nearly 3 weeks on.

It could be EDS as Jazzlover suggests because I’ve had some random bruising too and I’m fairly sure I have it. My neuro PT confirmed that I’m hypermobile the other day. But as you know the business of getting diagnosed for this would be very hard for me. Vasculitis would be hard too but at least it would help me access further treatments. Whereas I am no longer as prone to rashes and allergies as I used to be and already take antihistamine daily so I think Mastocytosis is a less good fit than Vasculitis.

I had all but given up wondering about the blood in pee really but my symptoms don’t tarry with intersistial Cystitis as I don’t have burning or discomfort when I pee and my cystoscope was very healthy a few years ago when the blood in pee was first observed by new GPs. I’d already had it for years though. It seemed to only clear away on highest dose of Cellcept. I know this because I had a UTI with Leucocytes but no trace blood at that time.

When the groin and pubic pain and feeling of swelling started a few months ago I was diagnosed with vaginal e-Coli infection and thought once this cleared the pain would go away. But it didn’t and it feels as if it’s in my skin and I want to rip off my underwear and pants - even when they are loose - just the touch of clothing hurts me a lot when this pain is bad - usually starting afternoons onwards.  This is why my GP thinks it’s SFN related and isn’t anything to do with blood in pee - just coincidence.

However I’ve noticed an increase in blood and lower back pain do seem to go together. And it all seems to interconnect with my erratic bowel function and related discomfort. It’s all rather distressing I must admit - very similar pain to my burning mouth/lips and gums and occasional Trigeminal Neuralgia if I’m ill or cry.

It is the spots and still not feeling that Sjögren’s fully accounts for everything that makes me think of small vessel Vasculitis underlying. Whereas you have PsA to explain for some of your extras but I don’t. And my very elevated inflammation markers also seem to fluctuate much more than they do for most with Sjögren’s whom I’ve asked. I could be wrong but my instincts are usually fairly good. The UK Sjögren’s expert was adamant that CRP is usually normal in Sjögren’s and Lupus - something to do with the liver she explained - can’t recall what. Mine has been increasingly raised again - not soaring but for me this shows something other than viscocity is going on.
« Last Edit: August 13, 2018, 10:43:07 PM by MAT51 »
Hashimoto's, seronegative RA, Primary Sjogren's, small fibre nld polyneuropathy, hypertension, IBS-C, GORD, BMS, highly allergic disposition!

Tiff123

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Re: Anyone have small vessel Vasculitis with their Sjögren’s?
« Reply #8 on: August 13, 2018, 10:32:24 PM »
New here, but this thread is up my alley big time.

Been diagnosed with Sjogren's several times via testing. My antibodies for it are through the roof! I have dry-ish skin all the time and dry-ish eyes but I don't have the typical brutal suffering for it (at least yet?).

What led me to get testing was developing serious purpura rashes all over my legs two years ago. Huge patches of that crap that would come and go. Often they were monthly on the dot, right around my period. Most doctors screamed vasculitis but even my skin biopsy would not conform typical vasculitis. :o Most thought I was allergic to something.

I had tried to eliminate various medications, nothing had worked. Tried gluten free, nope made it even worse. The food thing never seemed to help.

I had gone into remission for months but still would get little red bumps all over my shins and ankles off and on.

It came back with rashes on my legs and thighs (in patches) again in February and sometimes around my belly button and lower stomach and even on my left arm.

Had a few one week courses of prednisone too, it helps when I am on it but when I go off I am back to square one. My doctor won't let me go on it very often anyway.


Unlike you, I never had blood in urine. Urine was always tested fine. Kidneys fine, all internal organs fine. Have had a million blood tests. Elevated ESR rates are usually all I get that go up and down in waves.


Just diagnosed with sjorgren's and continuous mini blood rashes and occasionally swelling and bruising in feet and ankles.

Only thing I ever found to help work to an extent and lessen symptoms and even put me into remission once in two years was systemic enzymes. So I just went back on them two days ago hoping it would help again.


Just very odd. I can't figure out the correlation between sjogrens and these petechiae/pupura rashes that come and go and have been my #1 symptom. Nothing in blood tests have ever come up that screamed to any doctor "serious!". My skin is very fair so sadly any of these blood spots stick out big time. I wear pants all year round.


Hi and welcome!

My spots sound less significant than yours I must say. And they literally don’t itch or hurt at all - not even slightly. However I do get little spots around my face that also cluster in lines and itch slightly as they form and are non blanching too. I’d say it’s far from my worst symptom though - the painful neuropathy, weakness, tremors and IBS-c/ dysmotility  problems are my worst symptom by far.

Keep posting on the main forum and follow this post for suggestions from others here though.

If you ever get major purpura patches they can itch and burn for a few days until they settle down. A high ranked rheumatologist told me I likely had leukocytoclastic vasculitis, which can sometimes go away on it's own over time or just pop up now and then. He had also said any medications were unlikely to be the cause, it's just a mysterious thing. I notice it can come back in hot humid periods in weather and I am better in the cold (I live in New England). Me and hot weather do not mesh.

I am 38 now, it all started when I was 35/36. I used to think hormones can be tied together with all of this but male doctors often don't seem down with it.


Just to clarify, when I am in remission with the vasculitis rashes my spots really don't often itch or hurt at all......since they become very minor and small here and there. It's just those major purpura rashes I had initially that burned like heck all over my calves and feet.

Most of the time now I just get small patches or speckled spots here and there, looks like razor burn now at times or ingrown hairs. Never got them on the face or upper body.


Your symptoms though to me do sound like a vasculitis of sorts, it can hit people in different ways with symptoms. Your bladder and bowel issues, it's all inflammation of some sort.

Intelife

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Re: Anyone have small vessel Vasculitis with their Sjögren’s?
« Reply #9 on: September 17, 2018, 05:30:39 AM »
Hi All, without having looked up old threads on Vasculitis, I started a new one today

I am just finishing up one week of prednisone. PCP wanted me to take 60 mg daily for a week, but I got very hyper so just took 40 mg daily.   Even so, I still see one more  itchy painful bump on my pointer finger.  It started two weeks ago after a horrible year filled with Shingles for four months and Pneumonia for the ladt two months.  I had just started Plaquenil, but got off of it fast when the Pneumonia hit. 

Fast forward, I was presumptively diagnosed Zprimary SjS although the early biomarkers test was completely negative on all counts.

During the Pneumonia, my PCP said I had the highest SED rate he had ever seen in 30 years of practice.  .

I feel that with the dry eye and mouth issues pluses swollen parotid glands on right side, I have been SjS positive for at least six years, but had a lot of indicators including Mono, EBV ,HSV, HPV,, Reynouds, and just about every other virus known to man except Ebola.

So, I guess, I'm just open to learning all I can here so that I can be my own best advocate with my Docs.
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bluegardenia

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Re: Anyone have small vessel Vasculitis with their Sjögren’s?
« Reply #10 on: September 17, 2018, 04:51:08 PM »
MAT 51, your discomfort in the pelvic area could be vulvodinia, I had, sometimes still have it and it was terrible. but you can do something for this if it is this.
since some months I have awful pain under my feet that sometime comes up and affects also the pelvic area, I feel like if I had cystitis that I do not have, I have the sensation that I did not empty my bowel but I recognize vulvodinia. only that now it arrives from inflammation  of the nerves under my feet
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MAT51

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Re: Anyone have small vessel Vasculitis with their Sjögren’s?
« Reply #11 on: November 16, 2018, 03:32:58 PM »
Thanks - I seem to have mostly got it quite well controlled for now with help of coconut oil used lavishly as moisturiser
Hashimoto's, seronegative RA, Primary Sjogren's, small fibre nld polyneuropathy, hypertension, IBS-C, GORD, BMS, highly allergic disposition!

rapnzl

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Re: Anyone have small vessel Vasculitis with their Sjögren’s?
« Reply #12 on: January 05, 2019, 07:30:42 PM »
Greetings,

After 9 months with this purpura-like rash on my shins, it finally hit me to search a correlation to Sjogrens (DUH....nothing I did at home helped, I should have considered something auto-immune ::) ) Soooo I was THRILLED to find this thread!

Prior to posting here, I did come up with a few articles suggesting a correlation:

https://www.medicalnewstoday.com/articles/233747.php

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5031830/

My rash resembles ITP (ideopathic thrombocytopenia purpura) so closely that I began to wonder if I had leukemia! (Oncology nurse for many years....we always think everything is cancer.) Rare itching, no pain...just ugly.

The really odd thing is that the rash almost completely disappeared during the summer....when I wore shorts! From what I read, exposure to sun is most often what precipitates the rash, or makes it worse. That said, I am soooo relieved to find others in the same situation. Granted, I don't have a formal diagnosis yet, but the unconquerably dry mucous membranes, constipation, lack of ability to sweat....it can only be Sjogrens.

As soon as I can get enough time off work I plan to visit my PCP and get to work on this.

May you all find peace and comfort.

rapnzl

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Re: Anyone have small vessel Vasculitis with their Sjögren’s?
« Reply #13 on: May 19, 2019, 09:10:01 PM »
I neglected to post regarding my improvement.

Within 2 weeks of starting pilocarpine, the rash disappeared. Completely. I wish my other symptoms would improve half as much!!!!

vrystaat

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Re: Anyone have small vessel Vasculitis with their Sjögren’s?
« Reply #14 on: May 21, 2019, 09:50:09 AM »
All I have is very painful finger tips due to vasculitis (my Rheumatologist informed me).
My hands are also very cold. Nothing helps.
Sjogrens;Polymyositis;Polyneuropathy;Gastritis;GERD, Autonomic Neuropathy, Neurological complications, Trigeminal Neuralgia,Gamma 3 globulin low;Multiple infections;Brain fog; Ocular problems - blepharitis, scleritiis, dry eye,severe eye pain. Possible Inclusion body Myositis.Currently Endstage