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Systemic Vasculitis

Managing systemic vasculitis flares and seeking help

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How systemic vasculitis behaves - including flaring, remission, relapse
Recognising systemic vasculitis flares and seeking help
 

How systemic vasculitis behaves - including flaring, remission, relapse

People described their vasculitis behaving in a number of different ways after they had started maintenance treatment. Sometimes we heard vasculitis could be partly controlled but still active.
 
 

Grant has noticed his vasculitis starts “grumbling” when he is due another rituximab treatment.

Grant has noticed his vasculitis starts “grumbling” when he is due another rituximab treatment.

Age at interview: 34
Sex: Male
Age at diagnosis: 31
AAV (GPA)
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Because you talked earlier about it “grumbling”.

Yeah. Yeah, that’s the best way I would say it, to describe things and, again, I’ve kind of even been on, like, I’m saying family holidays, just to kind of give the aspect that it’s not like it comes on like a light switch, you know what I mean, it’s always there, I’m always kind of wary of it but when I say the “grumbling”, that’s when I feel things are getting that little bit, and at times, even if it’s in-between kind of doses of rituximabs, within the [inaudible], I’ll speak with them, they’ll say, “Maybe up your steroids a little bit,” and that, nine times out of ten, seems to just, well, like kind of, well, what’s the word I’m looking for? Like kind of, not mask it, that does just kind of give you a little bit of just, what’s the actual word, like, it does help kind of temporarily, but again, I don’t think they like you being on, as a rule, too much steroid. So, they just say, “Go up them until maybe hopefully just…” Nine times out of then, it is around about the time when I’m due. It’s almost like your body knows now, I’ve only had it a few times, knows your needing a rituximab.

Whether - I don’t think it is all just in your head, literally, but I, I do actually believe it’s like, not when you’re getting it, it’s, you do feel like – well I certainly do – thinking it’s almost like your body’s telling you you’re needing it now, you know what I mean? The rituximab, so. You ken maybe a few weeks prior to the, that’s when the kind of grumbling comes on, it’s like, “Well, okay, I’m feeling it,” and that’s when they maybe say, “Go up the steroids just to kind of give you a little bit of release until that,” basically, that’s, that’s what they do. But, but “grumbling” is the best way to describe it, because -

But again, it’s never been as bad as it was pre, pre, apart from maybe that last couple of, last month or so, just because it was delayed. That was as close as it has been. But again, never as bad as it has been prior to getting diagnosed.

 

Wendy’s doctor helped her spot the difference between her vasculitis flitting, flaring or relapsing.

Wendy’s doctor helped her spot the difference between her vasculitis flitting, flaring or relapsing.

Age at interview: 56
Sex: Female
Age at diagnosis: 53
AAV (GPA)
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The same doctor, I talked to him about, “Well what’s the difference between flaring and relapsing? I don’t know what’s happening with me,” and he said, “Well, the relapses are the big, big, big ones where we get all, you know, do things fast. The flares are what we try to control, the flares, so they don’t turn into relapses.” But he said, “But you have flitting as well,” [laughs] and the flitting was this idea that – and that’s how I make sense of it, like a butterfly, almost. It flits from one bit of my body to another. And that has been so useful, so I feel it flitting around, doing different things to me.

And then when it starts flaring, like when my eyes were going red before Christmas, that would be a flare. And then because I started having lung problems as well after Christmas, we are, are we heading towards the relapse, so I got very frightened. But then you think, “No, it’s okay, it was a flare and now we’re probably back to flitting again.” So yeah, [laughs] yeah, it’s really helpful, really, really helpful. But he’s the only doctor I know that talks like that; none of the others do. So, and I think, yeah, he’s a real specialist in vasculitis, I think he, you know, really evolved that language, to be honest, through lots of experience. And maybe – I always think that maybe he knows who he can, you know who needs, who needs, who needs the poetry and who doesn’t, and that can, you know, for me it’s very powerful and helpful, so hmm.

 
Other people felt that their vasculitis was well controlled.
 
 

Peter’s vasculitis has been “pretty well stable” for two years now.

Peter’s vasculitis has been “pretty well stable” for two years now.

Age at interview: 67
Sex: Male
Age at diagnosis: 50
AAV (MPA)
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Since 2017, all has been well [laughs]. My vasculitis has stabilised, there’s been no other problems really come along to concern me or, or the consultants. Nothing else has reared its ugly head. Nothing else has reared its ugly head, and I’ve been pretty well stable ever since, my consultants are very happy with me. That’s two years now, over two years since I’ve been like that. I only go and see the consultant every quarter now, and there’s very little we do, we spend more time talking about the weather and our holidays than we do about vasculitis [laughs]. Sorry, maybe I shouldn’t have said that.

 

Karen views herself as “in remission” from vasculitis (“Churg-Strauss”) and is happy to keep taking immunosuppressants.

Karen views herself as “in remission” from vasculitis (“Churg-Strauss”) and is happy to keep taking immunosuppressants.

Age at interview: 58
Sex: Female
Age at diagnosis: 53
AAV (EGPA)
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Yes, I suppose, so for me I suppose being in remission came when my bloods had been normal for, for sort of three or four months, also when they reduced my heart tablets and I was signed off by the cardiac area of [the large teaching hospital]. So that was about a year and a half, a year after everything happened. And it might’ve been around that time that it triggered the fact that I wanted to get fit and healthy and lose weight.

But yes, it’s almost like looking at a graph and you’re bobbling about and then suddenly the line comes down and it starts to go straight, and that’s I suppose the way I would visualise it. And yes, on a couple of occasions there’s been a little peak where I might’ve had an infection or, you know, there’s something of concern, there’s always a concern that the drugs I’m on can affect my kidneys, so they watch that closely but that’s not a problem, it’s never been a problem.

I think as well, I’d rather view myself as being in remission, I am very happy to continue the drugs I’m on. I know a lot of people really want to try and get off the drugs they’re on. I recognise the fact that long-term steroid use, we don’t really know how it could affect anyone, but I take the view that I’d rather have 20 years of active, fun life on steroids than try to get off them and feel ill or even worse, put myself in the position where the Churg-Strauss could come back. So, I’m not worried about taking the drugs and I’ll continue to take them.

So what drugs are you on at the moment?

The key drug I’m on is called azathioprine, and that was one of the drugs that was highlighted, that caused me to shield when we had lockdown, and the other drug I’m still on, five milligrams of steroids a day. Then I take one heart tablet, and then various other tablets to, you know, strengthen my bones and look after my stomach and all the rest of it, and I’ll take painkillers from time to time if I need to.

So yeah, it’s a handful of tablets every morning but I don’t, that doesn’t concern me. I’d rather take them and feel like I do.

 
We also heard that vasculitis could attack again, and that this could include new areas of the body.
 
 

Brenda “knew immediately” that her vasculitis was relapsing, as the symptoms were the same as before.

Brenda “knew immediately” that her vasculitis was relapsing, as the symptoms were the same as before.

Age at interview: 75
Sex: Female
Age at diagnosis: 69
PAN
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And I was absolutely fine then, I was still on the steroids, still on the immunosuppressant drugs, and they decided to reduce the steroids and take me off them.

And they took me off them after three years and I had a relapse. But once I had the relapse, I knew what was wrong. Because how I know now is, my temperature spikes – I forgot to say in the hospital, every day my temperature went up at lunchtime. Went high at lunchtime. It wasn’t there in the morning, went up at lunchtime very high, and I would night sweats. I would have gone through four or five nighties a night, just absolutely saturated. And that had been going on from the April. So, I’d had six months of that, every night, and this spiking temperature.

And when the spike in temperature spiked, I was really, really unwell, but when I’d get up in the morning, because the temperature had gone down, I was feeling all right. So, when I had the relapse, I knew immediately what was wrong because it started with the spiking temperature, it started with the night sweats, feeling absolutely really unwell, couldn’t get up the stairs. And I went to the doctor, and he told me to go home and take a paracetamol and stay in bed.

So, I then did that for about a week and then a doctor gave me an antibiotic for another week, he said, “Try this to see if this works.” I knew it wasn’t going to work and took the antibiotic for a week and I was really, really ill. And I phoned the hospital to see if I could get in touch with the consultant, but I couldn’t, so eventually this one doctor said, he said, “Look,” he said, “I think we’re just going to have to hit you with a high dose of steroids to see if this is going to…” I had reduced my steroids down to about five, and he said, “Look, we’ll hit you with a high dose and see if this works.”

And it did. The next morning, I could get up and go for a walk. It was so sudden. The high dose of steroids just completely made me well and I was able to go for a walk the next morning. So afterwards he said, he said, “We just got this in time.” But that was after a month of being told it was a virus, just go to bed with paracetamol, take this antibiotic. And I knew from the beginning what it was.

So, thank goodness, from that date, I went back then to the rheumatologist, and she increased my immunosuppressant drugs. And she said I will be on those for life, and I will be on the steroids for life. I am not to come off them.

So, I’m on low dose of steroids and quite a high dose of immunosuppressant, and I’m on blood pressure tablets which, thankfully, now have, you know, calmed my blood pressure. So, you know, that’s three years ago and I really am feeling well.

 

Pete was treated for vasculitis affecting his ear, nose and throat, but it went on to attack his kidneys.

Pete was treated for vasculitis affecting his ear, nose and throat, but it went on to attack his kidneys.

Age at interview: 67
Sex: Male
Age at diagnosis: 59
AAV (GPA)
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Fortunately at that stage I got a, a quick referral to the rheumatologist at, in [large city] and, the rheumatologist, they had me admitted to hospital within about two days, and for the next four days I received extensive, testing of all my major organs - kidneys, heart, lungs - anything you can think of, it was tested, and they determined at that stage that the vasculitis was confined primarily to my ear, nose and throat, and consequently they medicated me with a medication called methotrexate.

Now at that stage I was actually quite happy because the symptoms had all disappeared and my hearing had improved that I could get hearing aids, so I was leading a normal life apart from this methotrexate. So, this was in the summer of 2012.

After about four months on the methotrexate, I started to feel fatigued again, I felt lethargic, I just felt run down, and I just thought it was just symptomatic of the condition and the medication. Unfortunately, it culminated in me contracting pneumonia, which resulted me getting shipped off to the hospital in the back of an ambulance and straight into intensive care, because what had happened was the vasculitis had attacked my kidneys big style.

So, I was in intensive care at [acute teaching hospital in large city], pretty much I was out of it, didn’t know what was happening. But over the next ten days I received, I was on blood transfusions, I got, plasma transfer, I got kidney biopsy, I got cyclophosphamide, which is a pretty nasty medication, and I was getting kidney dialysis as well. So over that space of ten days I was really ill, but you wouldn’t believe it that after ten days they said, “Well your kidneys have recovered sufficiently and we’re happy with your progress and we can discharge you.”

Recognising systemic vasculitis flares and seeking help

People told us it was important for them to understand their own vasculitis and to recognise when they were having a flare. It helped that sometimes the symptoms were similar to those they had experienced before they were diagnosed. Peter said he went back to having around 75 per cent of his initial symptoms within the space of two weeks; “Oh, I knew straight away, yeah.” However, vasculitis symptoms are varied and unpredictable, and people were not always sure.
 
 

Although the symptoms were similar, Steve was too ill to recognise his vasculitis was flaring badly.

Although the symptoms were similar, Steve was too ill to recognise his vasculitis was flaring badly.

Age at interview: 60
Sex: Male
Age at diagnosis: 59
AAV (MPA)
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What I have been told, and I’ve also been told this is quite common, is that the reason I didn’t join the dots, and you know, recognise the symptoms as one, one complete set of serious symptoms, the reason I didn’t do that was probably because I was in fact very short of oxygen and my mental functioning was impaired, and I just wasn’t thinking straight.

And that’s what, what I mean when I say I really let myself go you know, my, my personal hygiene went down the drain, and that is just so unlike me. I’m usually extremely fussy about that. Well, you know, people think I’m really obsessed about it, and it just went, and in fact it took a colleague at work to like sit me down and say to me, “There’s something seriously wrong with you and you need help”, and that was in fact the day before I woke up in the early hours and could barely breathe. So, it just came on very, very quickly.

I think I just wasn’t thinking straight. I look back at work I did in that period and it was, it’s awful, I don’t know what I was thinking. I’ve had to re-do it all. So, you know, I think the balance of my mind was affected by, frankly, just by a lack of oxygen in my blood.

 

Holly has had to learn the “quirks” of her vasculitis to know when to seek help and when to wait and see.

Holly has had to learn the “quirks” of her vasculitis to know when to seek help and when to wait and see.

Age at interview: 24
Sex: Female
Age at diagnosis: 19
AAV (GPA)
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It is difficult day to day to get a grasp on what sort of, what your symptoms are in terms of what they actually are, you know, is it the vasculitis? Is it the medication that you’re on for it? Is it this other thing that you have wrong with you? Is it, or is it just because you slept funny that night and your arm kind of hurts? You know?

It’s one of those things like well we’re not quite sure and it is difficult, you know, sometimes a lot of the symptoms that you get, it is sort of a wait and see situation in that you know, you’ve got to kind of sit with it for a bit and go, “Ooh I’ve got a bit of a cough, is it, have I just got, have I just picked up a bug?” and then you’ve got to wait and see, you know, you wait a few days and see if it gets worse and that’s difficult as well when you have an autoimmune disease, especially when you’re on steroids and immunosuppressant therapy, because sometimes you can’t wait and see, you know, sepsis for example is a really big thing with when you’re on steroids, is a thing that they’re always telling you to look out for is, you know, getting an infection and then leaving it untreated for a few days when you’ve got an autoimmune disease can turn to sepsis real quick, and obviously, you know, that’s something you don’t want to play around with, but it does make it difficult in that, you know, you don’t want to burden the doctor every two weeks with this new ailment which sometimes disappears after a few days, but then sometimes it doesn’t disappear and you know, it is difficult to determine whether something is something you actually need to seek further medical help for or not.

And I think that’s one of the things that you learn when you get diagnosed with something like vasculitis is learning how to self-care and self-manage your own disease because you can’t be contacting, well I mean you could be contacting the doctors every, every week or two weeks, but it’s just not realistic, you know, and so you kind of have to learn the “quirks,” as it were, of the vasculitis and the quirks of the medication that you’re on and things like that, and sort of assessing yourself, obviously if it’s a very, if you’re very unwell then you should seek further medical help, but there are some situations in which it’s sort of like, “hmm, do I need to go to the GPs for this?” because it’s, it’s learning how to do that and learning whether it’s actually just the vasculitis, if it’ll go away in a few days and it’s all fine and I think that that only comes with time really, is having the disease for a while I know so much more now four and a half years down the line than I did obviously when I was first diagnosed in the first year, in the first two years, in the first three years, and I know when to, I know when to bother the GP with something or not or whether it can wait for the consultation that I’ve got in two months’ time or something like that.

 

Melissa gets flares of vasculitis (Behçet’s) and infections. When they come together, it can mean “a hospital job.”

Melissa gets flares of vasculitis (Behçet’s) and infections. When they come together, it can mean “a hospital job.”

Age at interview: 22
Sex: Female
Age at diagnosis: 18
Behçet’s syndrome
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So, a flare is sort of an exacerbation of my Behcet’s symptoms, so that can be to varying levels. Sometimes it will just be a couple of mouth ulcers, some very sore joints and a lot of fatigue, and then, which stops me doing a lot, but I can still sort of get on with my uni work from home. I still eat relatively normally. And then a, I get a huge flare sometimes that will completely stop everything, so the entire inside surface of my mouth will ulcerate, will start going down my throat, will come out on my lips, I’ll get loads of genital ulcers, I might start getting some skin lesions. So, flares are very variable.

And then the infections usually come about, sometimes they come with flares because I’ve become run down or more directly because an ulcer has got infected. And again, very variable, sometimes it’s just a matter of having a week of antibiotics and I’m still relatively able to do things, and then other times it’s completely debilitating, and I end up in hospital.

So, they’re both very variable. I’d say the worst time is when they come together, which happened when I hadn’t had the adalimumab for a month, I got the worst mouth ulcers I’d had in a very long time. I’d taken a lot of steroids to try to control it, and a lot of topical steroid stuff in my mouth, and that led to oral thrush, which made the ulcers worse. And then on top of that one of the larger ulcers on my tongue went into a bacterial infection as well, so I sort of had this triple whammy of having a flare up, a fungal infection and a bacterial infection, and that was really tough because they gave me a huge amount of antibiotics to try to keep me out of hospital.

But it was sort of this constant fear of, “Is it going to get worse tomorrow?” and sort of the moment that it got worse I knew it was going to be a hospital job, but it, it stayed at a fairly constant level, but the amount of antibiotics I was on I just had a permanently upset tummy, and that leads to inflammation in itself.

 
Isabel and Isabella were among those who had been told by their doctor how to recognise a flare and what to do about it, and Pete’s most recent experience was, “I could feel the flare coming back again - and phone call, and I was in there getting treated.” However, Dean and Brenda felt they weren’t listened to when they asked for help.
 
 

Isabel has giant cell arteritis (GCA), a type of vasculitis. She was told what a flare would be like and what she should do about it.

Isabel has giant cell arteritis (GCA), a type of vasculitis. She was told what a flare would be like and what she should do about it.

Age at interview: 76
Sex: Female
Age at diagnosis: 74
GCA
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What if something happened? What if, I don’t know, what if you got a terrible headache again? What would you do at that point?

If it was a terrible headache, I would monitor it for a short time to see what it was, and I wouldn’t wait too long. Because, at the beginning, before I got to the hospital, the GP said that if I got anything like that or disturbance in my eye or anything like that, if the surgery was closed, I’d to go to A&E. That bit – I forgot it, that bit, that was way at the beginning. At the beginning, they told me to do that.

So, you were quite clear, even at the beginning, that this had the potential to be a medical emergency.

Yes. I was told headaches, eyesight, jaw, sort of if the, I’ve not really had much bother with that, but I was told if anything like that happened, I’d either to get the doctor or to go to A&E. I’d forgotten all about that, sorry.

 

Dean coughed up blood again. This was a sign that his vasculitis had relapsed, but a hospital doctor wanted to send him home.

Dean coughed up blood again. This was a sign that his vasculitis had relapsed, but a hospital doctor wanted to send him home.

Age at interview: 47
Sex: Male
Age at diagnosis: 40
Hughes-Stovin syndrome
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But only a few years ago, again, I was just, I was doing nothing, and I coughed up blood, so you goes back to the doctors, and the doctor, you know, have to go back to the hospital. Now this one doctor, he said, “I’m going to send you home.” And I thought, “I’m not having this, I’m not being sent home, he’s not even checked where this blood’s come from and what’s going on.” You know, especially when I’ve thought, you know, only a few years earlier it, hang on, the start off was a little bit of blood to nearly six pints of blood coming out of me.

So, I sort of forced his hand and said, “No, you, no, I’m stopping in, I’m not going back as an outpatient, you’re going to contact [hospital in other region], you’re contacting this doctor or this doctor, this nurse and you see what they say. It’s not you telling me what you think.”

 
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