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Vasculitis UK
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Is vasculitis life threatening?

My husband suffers with osteoporosis and osteoarthritis and this has got worse over the last 3 years such that he has had 2 spinal operations and now uses a manual wheelchair outside of the home. We went abroad just before Xmas and whilst I was sunbathing he was in thermals so I took him to the Dr when we returned. It seemed like we entered another universe for 8 weeks, several blood tests were done, MRI scans, ultrasound, CT scans and a kidney biopsy, I've never known the NHS react so quickly. Initially we were told that he had CKD and possibly needed dialysis and then we were called into a meeting with 2 consultants who told us that it was 99% sure that Vasculitis was the problem. He went onto give information out and leaflets, so hubby has got Systemic vasculitis primary they think, ANCA PR3 (don't really understand even after reading up on it what it all means long term). I feel since then that we're living in limbo. He doesn't really want to know what is going on whereas I need to know. He initially had a week of steroid infusions followed by an infusion of cyclophosphamide at the end of February. Since then he takes steroids every day, started with 60mg and has been on the managed reducing doses since, also he has cyclophosphamide infusions regularly, started fortnightly and then went onto 3 weekly. His immune system is taking a battering, over the last month they've been monitoring him weekly cos he's had virul infections showing up in his blood tests which have shown themselves in lesions on his back and front. He's on two types of antibiotics and water tablets as the swelling in his legs, ankles, chest and face was giving concern. His fatigue is getting worse, (I thought he could sleep before all this reared its head but now he sleeps most of the time), I've spoken with our GP and the consultant and they don't seem to believe me. I'm worried that they aren't telling me everything, no one has said that this is life threatening but equally they haven't said that it's not. Not sure if anyone can help me to understand what he's dealing with, I'm trying not to do everything for him as this makes him cross and I stand back as much as I can but it's so hard.....

Sorry for the long story but I feel very isolated.

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The most important thing is getting a diagnosis and commencing treatment, which fortunately your husband has begun.

Vasculitis has many variations (and names) dependant on the size of blood vessels effected. But even within the same type of vasculitis, symptoms and parts of the body effected can vary wildly. Vasculitis becomes more serious when certain organs are effected. The kidneys are one of these.

Once his infections have been resolved I would expect him to improve greatly. It sounds like he has kidney involvement, but as he's not on dialysis, that is good news. From my first diagnosis of vasculitis, it has taken 24 years before I required a kidney transplant. My kidney function would drop everytime I had a relapse/flare of my vasculitis. But during the periods of remission, my kidney function was stable. This was until my function got below 20%, then over a 3 year period it dropped to 8% and I needed a transplant, or dialysis if I hadn't had the transplant.

The main thing is to get your husband in to remission and keep him there. He is more likely to stay in remission longer than I did and have less (hopefully none) relapses. Understanding of this condition and new treatments have improved the outlook since I was diagnosed 24 years ago. I'm still here and don't plan on dying anytime soon!! But it would be foolish of me to say that it's not life threatening.

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Many thanks gilders. My husband's kidney function is varying between 18% and currently 34% but isn't stable as yet, every blood test shows a variation, when he was first diagnosed it was 26% and has been up and down ever since. I think he's finding it difficult to come to terms with as he doesn't nor has ever felt unwell just really excessively tired. I didn't say but he is 70yrs old and we had just started to enjoy twilight years together so I think mentally this illness is very challenging too on top of the osteoporosis and osteoarthritis. We've been told that he will have at least 10 infusions of cyclophosphamide and he's only just had no.4 and his condition is deteriorating quite quickly. We're lucky because we attend Liverpool Royal Renal Hospital and the team of consultants so far have been amazing.

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So sorry for you and your husband. Vasculitis is a broad umbrella term for many autoimmune diseases afflicting the blood vessels. The old term for a positive C-ANCA/PR3 result was "Wegener's Granulomatomis."

Before steroids became available in the 1960's, it was fatal.

But today, with steroids and other immunosuppressive drugs, it is not fatal, and. treatable. Some patients live totally normal lives.

Some forms of the disease are limited, and only effect certain organs, including the skin.

So don't worry about death; that is not an issue. His fatigue is part of the disease, and likely aggravated by the steroids which gets even worse when doctors "rush" pts. into tapering down the dose too quickly. Plz. check other postings about the very slow taper plan which causes less flare ups; a good number of pts. go into remission permanently.

Steroids take over the function of producing adrenalin made by the adrenal glands which hibernate while the steroids do their job for them. The fatigue is to be expected.

Any mobility and muscle joint pain OA and ,now this , is rough, The drugs should be helping pain; if not, speak to his doctor about an adjustment in his dosing and the addition of other painkillers. Avoid NSAIDS like Motrin, Advil, etc. because they will not help, and cause stomach ulcers or bleeding.

But for all intents and purposes, it is no longer a fatal disease. Also. be aware that lab results are not always accurate. Ask for periodic repeat tests. I was diagnosed in 2016, also with OA, but am fairly mobile, and much less fatigued as I worked my way down to 4mg. Now my C-ANCA panel is negative. As an example. When I drop down 1.5 mg, I will expect a return of fatigue for awhile. If you feel your doctor is rushing you down on the steroids too much, or too fast, SPEAK UP.

Of courses, we are all different. It doesn't sound like the doctors are holding back anything from you and they are aware of kidney implications. With the meds, and some physical therapy when he is stronger, it is possible, he may make a limited recovery, or a complete one. With these autoimune diseases, we all have good and bad days.

The main thing with this disease is that his lungs and kidney function be monitored; attack on those organs is serious, but there are people in this forum who have had this scenario, and are doing well nonetheless.

This forum is the right place to come for information and moral support. Yes, it can be isolating at first, but here on this forum, you will find many kindred spirits, folks to share advice and experiences. You will find answers to your questions here.

Good luck and best wishes. Keep us posted.

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Many thanks for your helpful reply, I've just read it out to my husband and his response was encouraging, I may even be able to get him to join this site himself eventually. He's having a good morning so far so is able to listen and process. We do have an excellent care team and hubby has regular blood tests that are assessed by the consultants and we are phoned with the results on a weekly basis. I think initially we both hoped that this was an illness that would be treated and then disappear, that hope has now been tempered and we are beginning to realise that we have to focus on the here and now for the moment. He's on 20mg prednisolone atm but this reduces to 15mg next week and his schedule shows that they are looking at a maintenance dose of 7.5mg by September. We have both thought that his fatigue is getting worse and if I'm understanding it right it's probably because his dosage is reducing, would you agree?

I'm sorry to hear that you have OA as well, it's difficult to know which is worse some days, but you have a positive attitude which I'm sure helps. Kind regards.

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Yes, I would agree that the fatigue is a result of steroid withdrawal and the disease itself. And it gets worse before it gets better because when you get in the lower dose range, the fatigue and other side effects are usually harder.

I am concerned about your husband being knocked down by 5mg may be too much too quickly. This is usually the trend with doctors today. They are afraid of the steroids' effect on bone mineral density and the risk of fractures. Others feel quality of life is a better criterion.

But many of us believe going slow, and never attempt to taper when you are feeling unwell, or under stress of any kind so as not to trigger a flare up of the disease.,If you look for it on the forum, there are posts on this forum's recommended tapering schedule.

If you follow PMR Pro and Dorset Lady, they have posted them several times, or sent a link. Also check PMR Austailia since steroids are used to treat PMR and their members discuss this topic. You can reach them by clicking on the icon of 9 little squares above.

I know at Johns Hopkins Vasculitis Unit in Baltimore, MD, their protocol is reduce by 2.5 mg. and hold at that dose for 3 weeks minimum.

I am down from 16mg to 4mg and have been holding there for a few months. It took me a long time to get there. I feel reasonably well, and I might just stay there. I do not like the weight gain.

Although I am heartened by your doctor's intention to get your husband to a maintenance dose and hopefully, he will adjust.

And yes, irratibility and mood swings are caused by the prednisone too; patients are all different and have different reactions. Good days and bad days are what to expect, and it's impossible to predict which makes planning difficult.

Good luck..

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Hi Christophene47,

ANCA associated Vasculitis is different from GCA/ PMR etc. As it’s treated with Cyclophosamide the Prednisolone can be tapered much quicker. Recent research indicates that it’s the steroids that cause the infections, there have been some small trials using only 2 weeks of Prednisolone with great success.

Caution is required when comparing 1 treatment regime with another.

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Having been diagnosed with PMR/GCA and GPA (WG); all in a 6 mo.time period, I am well aware of the differences among them. There is nothing unique about cyclophosphsamide being used with Prednisone to treat many autoimmune diseases without much distinction. Methotrexate, Azanthopine are used the same way. All of them can cause cancer.

Studies are frequently very flawed I'm sure you know.

One shoudn't generalized about the best way to treat each individual patient with some AI disease regarding tapering. I refused the cytotoxics, preferring to stay with Prednisone and a very slow taper.I was luckily informed of the risk.

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Informed?

I am afraid that nothing you have posted can be called informed.

“ All of them can cause cancer “ this statement is meaningless without context. All types of cancer, some cancers?

Cyclophosamide isn’t used to treat “ many auto immune diseases “ , it’s usually reserved for the rare, life and organ threatening variants. PMR pro and Dorset lady are very knowledgable about PMR/ GCA but AAV is a completely different Vasculitis variant.

GPA isn’t treated with Prednisolone alone especially with Renal Involvement. None of the evidence based clinical guidelines advocate that approach.

You are calling me out for “ generalising “ yet are promoting the generalised Prednisolone reducing regime for PMR.

Well conducted studies, such as the recently completed PEXIVAS which had a low steroid arm, aren’t flawed. This was a multi Centre, worldwide trial and was funded because it was without major flaws.

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I find your manner inappropriate for this forum. I am not engaging in a conflict with you. No need to respond.

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Hi I was told by the renal consultants that if you were diagnosed before the early 70s you would have died with Vasculitis.

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I think the date is 10 years earlier, but that's right, there was a time before steroids when W.G. was universally fatal, but those times are over, thankfully.

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I am sorry you are going through such a difficult time.

Vasculitis can be life threatening if it is diagnosed late ( when the damage to main body organs is too big and the patient isn't responding to treatment) or if it is not treated as it should.

I am far from an expert but I would say that your husband has ANCA associated Vasculitis, possibly GPA. I have this type (4 years since diagnosis) and I had cyclophosphamide and high dose of prednisolone to start with.

It is very important to get treated by doctors with Vasculitis knowledge.

Here's a link with information about ANCA. At the top of the page you will find the helpline. I suggest that you contact them and get advice.

vasculitis.org.uk/about-vas...

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Thank you very much, we are under a specialist team of consultants that come together under the Vasculitis heading, and also have a vasculitis nurse that we can call on so feel lucky in that respect. Sorry to hear that you were diagnosed 4years ago, but I'm feeling more positive as I read so many different posts where patients are still here years on so I'm not so worried that my husband will leave me anytime soon. Kind regards.

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Hi peep3. You have come to the right place. Ask questions if you are at all concerned. Your husbands fatigue could also be a result of the cyclophosphamide as well as the Vasculitis and the fear of the unknown. Most of us go through a period of adjusting to the fact that our lives have changed drastically and we will have to learn to cope with that. Your husband is very lucky to have such a supportive partner. It is important that he/you have an awareness of this disease so that he can manage it. The treatment damps down the immune system so infections can be a problem and it’s important to get help for these promptly. As Zoe69 said the Vasculitis uk website is an excellent source of information. I hope your husband starts to feel well soon. Yes have had Vasculitis for 9 years and I’m not planning on checking out yet.

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Glad to hear it Mooka 😄.

We just find it strange that an autoimmune disorder where your immune system is under attack from itself is treated via drugs which attack your immune system also.

My husband was diagnosed with shingles a few weeks ago, but after reading a lot of the posts on here I'm not convinced that it was/is shingles as I first noticed what seemed like black patches on one side of his back, one of the larger spots has still not cleared up but has a sunken crusted look to it, if it hasn't gone by his next cyclophosphamide infusion then I'm going to mention it again. His scheduled cyclophosphamide infusion was cancelled because of this and another virul infection that showed up in his blood.

I'm so pleased that I've found this site, just wish I'd found it around February, think then I wouldn't have spent the last 3 months worrying myself into a state.

Thanks for your reply.

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Hi,

It’s not your immune system that’s under attack in auto immune disease, it’s the one doing the attacking! It becomes over active and goes a bit crazy, in Vasculitis it attacks the lining of the blood vessels causing them to become swollen and inflamed. The blood vessels then rupture and cause damage. Think of the kidneys as a giant filter and the blood vessels as bits of filter paper, if the bits of paper become damaged then they can’t do their job which is why the kidney function falls.

The cyclophosamide and other medications calm down the immune system and stop it being overactive. They aren’t selective though which is why one of the side effects is an increased risk of infection, it’s unfortunate that impaired kidney function puts us at an increased risk of infection as well.

Can you tell the specialist nurse what you have posted here, it sounds like there are gaps in the information you have been given.

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Thank you Tynemouth.

Yep sounds as though I haven't understood correctly, but I haven't heard it put like that before, it does make more sense now cos neither of us could properly work out the link between CKD and autoimmune disease. We are going to see the consultant on 23rd so I'm making a list of things to ask him about, so we'll see if I come away understanding better the process.

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A list of questions is a great idea. Often we need repeated information over time, these are complex diseases that Dr’s find difficult to understand, never mind patients!

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Sorry to hear what you are going through. It’s tough. I have PR3 ANCA vasculitis (GPA/Wegeners granulomatosis) and osteoarthritis in many of my joints. I had cyclophosphamide two years ago and the fatigue was very difficult to deal with. As the infusions got more spaced apart it got easier. I have found it very frightening as vasculitis has changed my life and I think it’s important not to underestimate its seriousness. Friends and family have found it easier to understand why I had to have chemotherapy once they knew how serious it was. They also know to stay away if they have infections and viruses. I’ve grown closer to them because of their support. My osteoarthritis is less painful on high dose steroids, but I’ve been more prone to injury. Physio has been a great help to me, so don’t be afraid to ask for a referral if there’s problems with joints in months to come. All the best to you both.

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