Vasculitis UK
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New Member - Intoduction

Hello All,

My name is Nick and I have recently, last month been diagnosed with GPA. I had been feeling rundown after a short flu bug in December 2017, then mid-January had some joint pain that lasted a few days which went away with some ibuprofen. The following week I had some discomfort breathing that was progressively getting worse over the week. I saw my GP at the end of the week who suggested possible lung inflammation from a virus and suggested ibuprofen. The following week the joint pains returned and were cycling round my body lasting a few days at a time. Return to GP who took bloods to look for rheumatism and more ibuprofen. Rash developed on my feet and blood from nose the next week. Back to GP and antibiotics prescribed. During all this I was losing appetite, not eating progressively drinking less and sleeping a lot. Also had sore gums, bleeding and mouth ulcers. Back to GP more blood tests called and antibiotics on 19 March. That night I started vomiting and other unpleasantness. Got to A&E and was advised I had 10% Kidney function left.

I spent 2.5 weeks as an inpatient with rapid diagnosis of GPA. I had 7 cycles of plasmferesis. Discharged 4 April but back again for a few days a week later with an infection. I am 2 cycles in to an estimated 6 to 10 cycles of cyclophosphamide. Currently tapering down on steroids at about 10mg per fortnight currently taking 50mg daily (Pred). I have fortunately recovered some kidney function and they are now about 25% functional so no dialysis required. Current medical view is that they will look to keep my immune system suppressed indefinitely with oral drugs following chemo due to high risk of flare up potential causing further kidney damage.

In general I feel pretty good. I am not experiencing any significant side affects of treatments, yet, but wonder if that is because it is still early in the treatment process. My current impression is that managing the suppressed immune system may be biggest challenge for now and welcome any advice or experience other members may have.

Regards

Nick

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Hi Nick, sorry to hear you've been though all this and glad you're feeling better. I was diagnosed with GPA in January 2017 with similar but different challenges. Since then I've had cyclophosphamide and Rituximab and high dose steroids. I have had problems with side effects. Some were immediate: the cyclophosphamide made me very very sick. Some have taken longer to emerge. They seem primarily due to the high dose steroids and have affected my joints, eyes and stomach. But it is difficult to tell what's due to the disease and what's due to the drugs: I leave that to the doctors to tell me what they think now! Hope things continue to improve for you.

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Hi Ziggy,

I have recently been referred to hematology by rheumatologist to explain the answer to the exact question you have just raised; what symptoms, blood tests results etc. are caused by the disease or by the drugs?

Have a hematology appointment Monday. And will also have to see a cardiologist as well. One thing at a time.

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Wow really sad that you had to go through that, I hope that you will be on a mend soon. I am just wondering how quick did they later slowly take you down on the steroids was it after the treatment of the chemotherapy?

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Still taking steroids. Dropped from 60mg to 50mg after 2nd cycle of chemo then 40mg after third. Firstcthree cycles were two weeks apart. Now on 3 weeks between chemo and reducing at 5mg between cycles until I get to 20mg. Not sure what happens then .guess it will depend on blood tests.

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Right ok so they have lowered your dose of the steroids quite quick which is good. I think the treatment you are getting willl really help and then you will not need the steroids anymore. So overall how long have you been on steroids for?

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Started with three x 1000mg in hospital on diagnosis. So guessing about 21 March. Then on to 60 mg after three nights to 60 mg for 4 weeks 50 mg 2 weeks and now 40mg until 15 May when I will hopefully drop 5 mg.

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Okay that's good you went from a dose that high to 40. I was wondering did you have any ringing in the ears after your treatment more than there was before?

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No ringing in my ears. An area that seems so far to have been unaffected by the condition or treatments.

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Okay well I hope you are doing well and hope that everything gets better for you you've been through Alot

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Hi Nick

I have also been recently diagnosed - Feb 2018. I have my 6th cycle of cyclophosphamide tomorrow with another 6 to go. My symptoms were all like yours but my lungs have been badly impacted so waiting to see what the long term prognosis is.

My pred is now down to 25mg. Was at 70mg in Feb but have developed Cushing’s syndrome too!

Glad to hear you’re doing better. I am also the same - in between chemo I am back working at 80% capacity from home. Have to manage my fatigue but feeling very hopeful to get to being in remission (ANCA negative) by October!

Keep moving forward!

Carolyn

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Carolyn,

Thanks for the support and sharing your experience. hopefully I can achieve a similar working level.

Nick

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Hi Nick sounds like you have had a tough old time and i can understand as i followed a very similar path from 2015 to date.My consultant said to me recently Keith it is a long road you are on and been on but the way research has progressed into these issues we can control things far better,I have now got my brain engaged into what to eat and what to aviod to protect my kidneys as the were affected and now keeping stable at around 30-34 GFR.My steroids are now at a good level 5mg every other day which is good and been having Rituximab every 6 months for 18 months.Its all tough at times but talking on here and people in similar does help a lot i have a guy who was in hospital with me at the same time with same and i keep in constant touch and it helps a lot to talk how each other is going on.Stick with it Nick good luck.

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Thanks for sharing your experience and support. experiencing quite a bit of jopint pain post cyclophosphamide which isn't fun. Rheumotology are suggesting putting me on Azathioprine rather than Ruituximab. Any experience with the former?

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Retuxamab appears to be the most effective drug for many people, but it is very expensive and I think this the main reason doctors will put patients on cyclophosphamide followed by Azathioprine. For some people these two drugs might work. For me they only worked for a few months before I relapsed. I was given another round of cyclophosphamide and continued with the Azapthioprine, but a year later I relapsed again. My kidneys were hit badly each time and I am now on dialysis. I couldn't be given any more cyclophosphamide so I was given Retuximab, and continue on it with an infusion every 6 months. It has been 18 months now and I get my next infusion in June. I'm surprised (but encouraged) to read that some people have been on Retuximab for more than 5 years, because I understand that it has only been used as a maintenance therapy for about 2-3 years, so studies on its long term use are still in their infancy. I think it depends on your doctor what is prescribed for you, but I suggest you do as much research as you can on all 3 of these drugs and make your own decision. I got Retuximab the first time through my nephrologist, but she wasn't keen on me getting it as maintenance therapy, so she sent me to another nephrologist who told me Retuximab wouldn't be suitable for me because my Igg was too low, and he put me on Mycophelanate, which made me violently sick for 2 weeks. So I went to my Rheumatologist who told me a low Igg is normal on Retuximab because that's how it's supposed to work. So he got me on Retuximab as a maintenance therapy. But as others have mentioned to you avoiding infection is a priority when on any immune suppressant. I don't know whether Retuximab makes you any more vulnerable than Azathioprene.

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Sorry you have been diagnosed with GPA, Nick. It takes a while for all that it means to sink in. I was diagnosed in 2014, and symptoms were much the same as yours except for the rash and nose bleeds. I was hospitalised for 10 days being treated with antibiotics while steadily getting worse before finally being diagnosed correctly. Your kidney function may increase some more over the next few months. Relapse is a concern, though. Unfortunately, you don't have much control over that. Some people do better on one immune suppressant over another. If you do relapse, your doc may try a different one. I understand that the likelihood of relapse is linked to the type of antibody that you have In your system. I believe that shows up in your ANCA test. Avoiding infection while on immune suppressants is challenging. It's something I always think about when socializing and being in public places, especially in the winter months. I avoid even family gatherings if anyone is unwell, and during flu season I even avoid shaking hands with people. Washing my hands is something I do much more frequently now. You start to think about the things you touch when you are out in public where you are more likely to pick up a virus or bacterial infection (shopping carts, door handles, elevator buttons, etc.), so washing my hands is the first thing I do when I come home. I also always carry a hand sanitizer when I'm out. You become more aware about the things and situations to avoid when you are on immune suppressants. But only you know what risks you are willing to take.

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Thanks for the support and sharing your experience. Hoping to get a bit more organised with managing infection risks. Having to learn to be a litlle more strict with myself at least during the Cyclophosphamide phase of treatment I think.

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Hi Nick, welcome to the club. I have MPA but your story sounds very familiar, particularly the trips to the GP, getting no where and the cycling joint pain/swelling. The good news is you are responding to the treatment and the worst is over. You do have to try to steer clear of viruses, particularly at the moment with your immune system being heavily suppressed. I did end up with a gastric bug during the initial treatment but nothing too serious thankfully. Hopefully you have been told to get a pneumonia jab and a flu jab in the Autumn. During my six years on imunosuppressants I haven't suffered unduly with viruses (a bout of viral meningitis being the worst - unpleasant but not life threatening). In 2017 however I seemed to have a string of cold viruses that lingered. I discussed this with my consultant and we jointly agreed to reduce the imunosuppressants. It was taking a bit of a risk because I'm still ANCA positive. I am now just on 1000 mg of Mycophenolate and so far so good. As you will soon find out there are no two cases of vasculitis exactly the same, we are all different in terms of how the disease affects and our prognosis. Some can get off the maintenance drugs completely while other can not. Hope you continue to improve. Chris

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Chris,

Thanks for sharing your experiences it is really helpful. Experiencing bad joint pain after cyclophosphamide. Assuming this may be a regular side effect. Rheumatologist is suggesting Azathioprine as possible maintenance drug but got some way to go before then as only three cycles into a possible 10 cycles of chemo.

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Your vasculitis is not yet in remission so it may well be responsible for the join the pain. Azathioprine tends to be the preferred maintenance drug for ANCA associated vasculitis. Unfortunately I couldn't get on with it. However the Mycophenolate has kept me in remission for over 5 years now. Fingers crossed it stays that way.

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Hi Nic, I was diagnosed with GPA in 2007 and followed a similar treatment plan to you. After the six months of treatment I had no symptoms for 8 years! I was taking 5mg of prednisone and 150mg of Azathiaprine everyday but was well and symptom free for many years. The last 3 years have been more challenging though. I’ve had more cyclophosphamide, plasma exchange and, more recently, rituximab. I’m just waiting for my second infusion of rituximab. I seem to get about 8/9 months of good health before I need another dose. I’m also still on prednisone and cellcept. It’s certainly a challenging illness but I can honestly say I still manage to live a relatively normal life and are able to do most things I want to. Obviously, this illness affects people in lots of different ways. My main problem is lungs and joints. I think what I’m trying to say is there are going to be good times and more challenging times ahead, but a positive mental attitude helps me get through the tough times.

I had no real side effects with the initial treatment (except weight gain from steroids). Actually, I have just remembered, my children used to say I had ‘roid rage’ from the steroids in the early days! It’s amazing how one forgets things over time.

One thing other people have mentioned is how difficult it is to know if symptoms are from illness, medication or just age related.

I have only recently discovered this site and find it extremely helpful and informative. Hoping the coming months are not to difficult for you.

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Thanks for sharing your experiences it is really helpful. Experiencing bad joint pain after cyclophosphamide. Assuming this may be a regular side effect. Rheumatologist is suggesting Azathioprine as possible maintenance drug but got some way to go before then as only three cycles into a possible 10 cycles of chemo. Do you experience any obvious side effects from the Azathioprine?

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Hi Nic, once I started the initial treatment all of my symptoms disappeared almost instantly. I had very high dose intravenous steroids for a few days but I was put on cyclophosphamide tablets and prednisone tablets. I was living in Germany at the time and that’s how they treated GPA

I was on the tablets for 6 months then swapped to the azathioprine. I can honestly say I don’t remember any side effects of the drug. I managed to get the steroid dose down to 5mg per day and was in good health for years. It was very scary when I was diagnosed but I received fabulous treatment in Germany. I have also received fantastic treatment here in the UK. I had a second round of intravenous cyclophosphamide in 2016. I was put onto cellcept as my maintainance drug. My steroid dose has been up and down like a yo-yo since then! Hope this helps you and please ask any other questions which you think I might be able to help you with.

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Hi Nick,

I was diagnosed with MPA (Wegeners) which also affected my kidneys and lungs in 2015 and after being on Mycophenolate for the last 2 years I am now doing ok and the condition is being monitored by the hospital with ongoing blood tests ect.

Because you also now have CKD are they monitoring your blood pressure? because if you have raised blood pressure this can cause more problems for your already damaged kidney's. I had never suffered with blood pressure before I had vasculitis but since being diagnosed I am now on pills to keep that under control too!

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Regular monitoring of blood pressure and bloods in general as I am only part way through cyclophosphamide phase of treatment. So far blood pressure has remained fine. But experiencing bad joint pain the evening after cyclophosphamide.

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Hi Nick. I'm new to this too. I've had a better journey than you and consider myself lucky for that. Unfortunately yours and the other people who have replied, from what I've read on the vasculitis uk Facebook page, it's not uncommon. I suppose it's what comes from having a condition in the rare diseases list. I hope things move forward well for you.

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Thanks for reply and support hope all continues well for you.

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Hello Nick,

I think you are right; the biggest challenge is management of immunosuppressive medication; I am glad you are feeling pretty good; that is wonderful news considering what you have already been through.

I was also feeling very well on 16mg a day of Prednisone; I was/am positive for the PR3 antibody with severe upper body pain starting after 2 flu-like episodes. I also had a history of having had the chickungunya mosquito borne virus 2 years prior( a whole other subject) ; the prednisone worked fast and dramatically.

Doctor had me start tapering slowly after about 6 months. When I reached 8 mg a day, I temporarily lost vision in my left eye while calmly watching TV. In response to that incident, low dose oral methotrexate started and resumed original dose of prednisone; now am tapering again, now at 8 mg. Have just had duplex doplers of eye orbits and carotids, photos taken of back of eye , trying to rule in or out giant cell arteritis ; results pending; under care of neuro-ophthalmologist. Fortunately, the vision loss has not happened again.

He wants me to see a cardiologist as well. These diseases, GPA, GCA, etc. can flare up anytime, anywhere in the body. It does seem like these diseases ,may for some , be viral in origin. The chickungunya virus has been implicated as a trigger of autoimmune problems 2+ years after recovery from acute infection. Many rheumatologists who do not practice in tropical or sub tropical regions have no idea.

I do hope you continue to feel well and that your kidney function improves more and more, and that your taper goes smoothly.

Best wishes...

'

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Welcome Nick! Wow you just had to have all the symptoms didn’t you?! So glad to hear you’re feeling better and sounding POSITIVE - this is the key my friend, everyone is affected differently by GPA and everyone finds their own way of dealing with the condition but expect some ups and downs. Stay positive :)

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