I was diagnosed with SLE last year when I developed Hepatitis and came down with really bad headaches, pain and stiffness in my neck and shoulders rigor and other flu like symptoms,my Rheumatologist deduced that it was just that, 'an autoimmune Hepatitis' commenced me on hydroxy 200mg twice a day and it settled after several weeks of rest. I have since had severe headaches treated with propranolol and a bout of uveitis. My ESR and CRP are always normal even with the Hepatitis. I have now developed a swollen face worse on exertion tightness in my chest and hoarseness coming and going, so I did some investigating, and I think I may have developed autoimmune vasculitis. My Rheumatologist categorically dismissed my concerns and said the symptoms of vasculitis are " very specific" and I believe he said this because of the negative ESR/CRP. Does anyone have any experience of vasculitis and also have negative ESR/CRP? I have been unwell for so long now I fear I may lose my job and never feel well again but according to my Rheumatologist my Lupus indicators suggest all is quiet with it now.
Regards and Wellness wished for you all. X
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Sleepynellie
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Have you spoken to your GP about your concerns regarding Vasculitis?
Both Vasculitis and Lupus are similar in that they are both initially difficult to diagnose and share very similar symptoms. However, they are both separate diseases in their own right. Below is the link to Vasculitis UK which has a factsheet explaining about the condition in more detail.
If you think it would help, I can send you a copy of our new self help employment guide for people with lupus which offers advice and useful contacts in relation to the workplace. Just let me know your address by sending me a private message or an email, and i will pop one in the post for you. hayley@lupusuk.org.uk
Hope this helps and If you need anything else please do let me know.
Vasculitis is very likely to give a high ESR, unlike lupus that can give symptoms but not show in blood tests. I'm not sure why you think it is vasculitis, your symptoms suggest lupus, including the hoarseness and severe headaches.
Unfortunately there are two types of rheumatologists - those who believe that lupus isn't active if blood tests are fine and those that go further than blood tests and believe the symptoms. Yours seem to be in the former category, which doesn't help you when you're worrying about new symptoms.
I'd go back to your rheumatologist and ask to try a short course of steroids and see if your symptoms improve (very likely). After that you might want to ask for a stronger maintenance drug, an immunosuppressive of some sort, if the symptoms return. I would challenge his view about lupus being quiet when blood tests are fine. I have the same - all my blood tests are fine but I have flares nevertheless. I'm lucky that my rheumatologist is one of the second category, I.e. Believes the clinical presentation, not only the blood tests.
If you're still worried about vasculitis, ask on the vasculitis forum of healthunlocked, if you haven't already.
My apologies for this rather belated reply. I know I had written just after I first posted this question but sometimes i have difficulty getting it to post and it appears to have failed in this case.
Many thanks for your thoughts. In answer to why I think it is vasculitis and not lupus symptoms is mainly because my Rheumy said that my sore hands and feet are not lupus symptoms and that we may never find out what is causing my headaches ( my brain and cervical spine MRI scan all look normal). The palms of my hands and soles of my feet become sore and feel inflammed and a mottling effect appears on them and my forearms, add to that a total lack of energy a feeling of being quite unwell and the same stiff and painful neck and shoulders I began with which after several weeks now has extended to include my hips and lower back.
I admire your courage when you mention having to challenge a doctors view on blood results and symptoms I have not previously felt empowered to do so but I guess I just needed some support, which you have given me, thank you.
I have asked for another opinion now and am hopeful that I will be able to give my employer some answers with regard to my ongoing symptoms and relief from them so I may return to work.
Seems to me purpletop has given you a great reply to your question sleepynellie!
I've been wondering about vasculitis too, but, like purpletop, my bloods aren't revealing it. After 3 years on 400mg hydroxy, i still had certain sets of symptoms flaring. So, my rheumy & gp did exactly what Purpletop is recommending: prednisolone tapers. These were so effective in treating those lingering symptoms, that Rheumatology added daily mycophenolate to my treatment plan last January, which is even more effective for me than pred, and helping me feel better than I have in over 30 years. I'm on 1000mg myco daily, considered my therapeutic dose.
And, it seems my case is being watchfully waited on re vasculitis. So I did what Hayley has suggested, and have been relieved to find some versions of vasculitis are treated with myco
Hi Barnclown, It sounds as though you are well at the moment?. Thank you for taking the time to reply, am I right in thinking that you no longer take Hydroxy just Mycophenolate.
I've told my Rheumatologist that I am willing to try anything hence the Amytriptiline because she is suggesting possibly fibromyalgia, but she does concede that it doesn't explain my sore mouth and facial symptoms really. I think because in terms of disease management I am still very newly diagnosed it is becoming apparent that I will have to be more patient and better educated about Lupus and this site will be of immense help to me and so many others, Thank you so much.
Hello again & you're v welcome sleepynellie! My feeling is that you're well on the road to figuring out your version of all this...which, yes! does seem to often take some time....trial & error....collaborations with your drs + forum contacts...
The daily oral meds I take are: 400mg plaquenil (hydroxy) + 20mg amitriptyline (at night) + 1000mg myco cellcept. I also use various prescription topical steroids, antibiotics & emulsions etc
I'm relatively well, thanks....basically, am feeling more symptom free than before I began myco, but is life with our condition/s ever simple....3 weeks ago, I felt well enough to try a little holiday (the first real hol in many years) which I managed really well until the last 2 days when rough seas set off a cerebral/neuro flare...which my specialist lupus nurse & gp are helping me sort out....never a dull moment!
Vasculitis can show up in in skin and mucus-membrane with distinctive polygonal shapes : usually rhomboid (diamond) shaped , e.g. see corners of mouth on this image ... dermnetnz.org/common/image....
[ other mucocutaneous manifestations of vasculitis are possible ]
Thank you a_scot, you must have done quite a bit of research. The abstact re svv was of great interest to me.
I do not know anything about vasculitis, but I do have diagnosed autoimmune hepatitis, as well as diagnosed SLE. The only way to confirm a diagnosis of autoimmune hepatitis is by liver biopsy (as well as having anti-smoth muscle antibodies and abnormal AST/ALT readings etc), did you have these tests? Autoimmune hepatitis and SLE often go hand in hand. I am not a doctor, but this is what I have been told by my specialists.
I have similar symptoms to you with the rigor & flu like symptoms. It feels like my joints lock at times, which is very painful. I also get painful glands & fevers with this, while my ESR remains persistently low.
I was diagnosed with vasculitic lesions last year, & some clotting incidents prompted my rheumy to test for vascultis. The ANCA test was negative. However, as my symptoms were worsening, it continued to worry me that I might have more than just secondary vasculitis.
My local rheumy is very nice, but I often feel like I never get to the bottom of things with her. She has however regularly consulted a lupus specialist about my case, & I finally got to see this specialist yesterday.
One of my first questions was about the possibility of primary vasculitis & she was able to put my mind at rest about this. I'm only just beginning to digest a lot of what was said, but basically lupus often causes secondary vasculitis in many different areas of the body. But the overall disease picture is very different between primary lupus & primary vasculitis.
I hope this helps to put your mind at rest a little. But it is important that you address these symptoms with your rheumy. In the long term I would encourage you to request a referral to a lupus specialist.
Hello roobarb, your Rheumy Lupus specialist sounds like she has lots of experience of SLE and vasculitis I don't suppose she is in the North west area is she?. I'm sorry but I have another question you may be able to answer (well 2 actually). Do you only get a positive ANCA test if it is primary vasculitis and what was her explanation for your low ESR results given that you have clearly had some vascular incidents?. I know I appear desperate but that's because I am.
ANCA positive is neither here nor there - just because ANCA isn't present, it doesn't mean ANCA-vasculitis isn't present. Which makes everything so much more difficult to diagnose. Lupus-related vasculitis could still be present without ESR out of range. Primary vasculitis is more likely to show an elevated ESR.
Do go to the vasculitis uk forum - the administrators there are excellent and very knowledgeable, they can help much more on primary vasculitis.
Yes sleepynellie, it is in the north west! I was seen at the manchester royal infirmiry lupus clinic which is run by prof Ian Bruce.
I live in Chester so it was a long way for me to go in my current state of health. But it was worth it.
Re the ANCA tests, as others have already said, it is possible to have primary vasculitis with a neg test. But the symptom picture between the 2 is entirely different. The consultant basically said that I wouldn't have been sitting in front of her on Wednesday if I had primary vasculitis. And what I take her to mean is that the symptoms develop quite quickly into a separate entity from those of lupus.
Personally I found the vasculitis route map quite difficult to follow. It gives great info, but if you haven't got a diagnosis there is a lot of scary stuff on there. I did get helpful info & support when I posted on the vasculits uk HU site like this one, & also from a member who lives near me.
And re ESR, she said many lupus patients have low ESR even when they are quite obviously in a severe flare, & once again this is where the symptom picture is so important, & where a lupus specialist really comes into their own. Unfortunately for us the tests have not yet been developed which can show the subtle difference which occur in our bodies with lupus. This is why it so often 'flies under the radar' & it hard to diagnose.
It sounds like you are very concerned about the possibility of primary vasculitis. Please be assured that from everything I have read & been told I believe your rheumy really would know if it was this.
I know this doesn't help when you are suffering as much as you are now. Do you have a rheumy nurse helpline, they will know what is the best action to take in light of your worsening symptoms.
Big hugs & please keep in touch & let me know how you are. X
I think if you asked people with a primary vasculitis how long it took them to get a diagnosis ( there was a poll recently on the VUK Health Unlocked site ) then a significant number of them struggle with severe symptoms for over a year before being diagnosed.
I think those Rheumy's in specialised clinics in large cities can diagnose these kind of disorders and differentiate between connective tissue and vasculitic diseases much easier than a Rheumy working in a District General Hospital, for example. Even then some specialists can struggle as blood tests and biopsies can be negative or equivocal even when the disease is active.
The VUK helpline is excellent and run by someone who has great knowledge of vasculitis and works closely with all the top specialists.
I don't mean to be argumentative but my own experience is that Dr's sometimes misdiagnose or play down symptoms that they don't have an explanation for. Better to push for answers than end up with end organ damage. Your Rheumy is wrong Sleepynellie, sometimes the signs and symptoms of vasculitis can be very general. I would guess that most people would think that the signs and symptoms of a heart attack are very specific but many are missed due to an atypical presentation.
That's a good point keyes, that vasculitis can sometimes take along time to diagnose. I must admit I have limited knowledge of the subject & can only comment on my own experiences & hope they are helpful. X
thanks roobarb for this very helpful clear concise explanation of the diffs between primary lupus , primary vasculitis & vasculitis secondary to lupus...I need to understand this, but was feeling a bit "at sea" about it all...now am feeling better grounded! Am so glad your appt yesterday was constructive. xo
Roobarb wrote "I was diagnosed with vasculitic lesions last year, & some clotting incidents prompted my rheumy to test for vascultis. The ANCA test was negative. However, as my symptoms were worsening ..."
i.e. The skin lesions look like vasculitis , but it's a different mechanism of vasculopathy . ( the "pseudo" bit does not mean it is a benign look-alike : it is just as serious as vasculitis).
There is a blood test, and treatment for, "antiphospholipid antibody syndrome" , aka "Hughes Syndrome".
Hi Sleepynellie,
There is a very informative document on the Vasculitis UK website called the Vasculitis Route Map. It describes the various types of Vasculitis which are categorised according to the type of blood vessel affected ( small, medium and large ) although there are overlaps and of course secondary vasculitis.
Not all types of Vasculitis are associated with a positive ANCA blood test and even more confusingly some types which are ( ie GPA ) can present as ANCA negative in some patients.
CRP and ESR aren't always a very good indicator of disease activity and some patients never mount a strong inflamatory response.
As others have said the best Consultants are the ones that have built up expertise treating a lot of patients with these rare diseases and take into account a patients symptoms as well as blood and other markers. If you continue to feel unwell and aren't getting anywhere with you Consultant then ask your GP for a referral to a Lupus specialist. I know the clinic at Addenbrokes in Cambridge sees both Lupus and Vasculitis patients and is ran by a Dr David Jayne and team.
No one will care as much about your health as you do and sometimes ( unfortunately ) we need to actively seek information, referrals, scream and shout ( a little ) etc to be given the treatment we need.
Good luck
Keyes
I'm somewhat surprised by trying to separate Lupus from autoimmune attack on vessels if that's what the original poster is trying to say? Or Am I wrong to think this way? then I shall stand corrected. It's an excellent thread though, I'm willing to learn and be enlightened
I haven't read the whole thread except a few posts at the beginning.
Hi Omega, I'm Sleepynellie the original poster and what I'm trying to say is that my understanding of the mechanism that causes the inflammation affects the blood vessels that supply the involved organ . If that makes sense but I dont know if that is correct.
I hope you don't mind me adding to this thread so long after you posted but I couldn't believe how similar my own experience has been of late. I, too, have a low ESR (single figures) but hideous symptoms which very much feel vascular in nature (antiphospholipid antibodies negative so I suspect maybe vasculitis). My head, neck and shoulders/upper back feel like they have hot barbed wire in them (fibromyalgia discounted) and having been seen by several doctors of late, opinions vary hugely. The non-rheumatologists have suggested anything from hormonal migraine to musculoskeletal problems but rheumatology seem to have put it down to a 'flare' and have given me amitriptyline which has helped me sleep I think but not got rid of the horrible symptoms.
I have also had very red eyes, temple and jaw pain (one doc suggested temporal arteritis but without ESR difficult to diagnose) and systemic coldness. I've just spent 3 nights in hospital where I had a head scan, chest x-ray, eye tests (eyesight wasn't good throughout) but nothing was concluded. Best guess was that lupus was flaring as my malar rash was like war paint. It doesn't help when some of the symptoms are so difficult to describe and/or some doctors do not listen properly or dismiss you without offering any explanation. They do all seem so pushed for time!
Anyway, I wanted to say that this thread rang quite a few bells. I am off to a Lupus clinic on Monday where hopefully I will get more specific help. Sometimes, I think we should be able to spend half a day with Lupus specialists and that half an hour here and there is false economy as not all questions are answered and not all symptoms are discussed.
I would be interested to know how things progressed…..
I can't remember the times how many times my bloods have come back as normal, even during the height of a flare. I am still suffering after 6 years. Good luck on your quest.
You use the term 'Umbrella diagnosis', that is what I thought too about SLE but the Rheumatologist perhaps didn't understand what I was trying to say.
I know I haven't got Primary vascultis but having been told that my sore, burning hands and soles of feet were nothing to do with Lupus I was somewhat distressed
I now realise that in addition to my hoarse voice, gravel like lumpy neck,
( which has now shown on Ultra sound to be inflammation) that my sore tongue and facial swelling and pain is Rheumatic and is directly linked with the headaches and neck and shoulder and back pain the blurring of my vision and light headedness and the foggy brain. This I believe is small vessel vascultis, I can see the the red thread like vessel's very clearly on the underside of my forearms and have a degree of livedo on the top of them.
I'm on 5mg of Prednisone now and reduced Hydroxy chloroquine to 200mg a day, I really want to be on the right treatment, but should that be stronger immuno suppressant?. I am going to ask at my next appointment if I can try the next level as the Prednisolone is ok some days, but I still get breakthrough symptoms and I don't want to keep throwing more Prednisone at it if something else is more appropriate.
I do hope your treatment is working for you and that your health is improving. How long has it taken to get your diagnosis? .
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