Newly diagnosed MPA pANCA vasculitis with PBC anitibodies

[Clay6852]

Hello, thank you for allowing me to join the group. Until a month ago I had never heard of this disease. I went to the rheumatologist in 2016 with joint & muscle pain, dry eyes, facial sores, fatigue, cold sores on & in nose, inflamed salivary glands, to be told it was fibromyalgia.

Then 2019 I started to pee blood. I was referred to urology and went through all their tests which came up negative. They just discharged me, even though I had visible blood and protein in urine. I complained & was referred to Nephrology, who told me for 10 months I had IGA neuropathy. My kidney function kept dropping so I had a kidney biopsy 5 weeks ago. I was called back to hospital 2 weeks after and was told I have MPA pANCA vasculitis. The biopsy showed severe inflammation and crescents 😢 even though my GFR was 48 and CPR 4. The doctor apologised for getting it so wrong. I am now on 60mg Prednisone and infusions of cyclophosphamide. I’m finding this very overwhelming. I still work full time from home running a dog boarding establishment. Will this treatment affect that? I have no idea what to expect and a little scared. Your advice is appreciated x

[jane1964]

Hello I was diagnosed with MPO ANCA approximately two years ago after a prolonged period during which time the doctors didn't know what was wrong with me.My symptoms were severe episodes of muscle pain which eventually became all the time swollen joints and tendons shivery flu like symptoms and vasculitis sores around finger nails and tipsand a also had sores around my nose which no one could explain and no creams helped.My vasculitis had been treated with azathioprine and hydroxychloroquine which have really helped me.I also have primary biliary cholangitis which was diagnosed 10 years ago . Interestingly the azathioprine appears to have helped my liver function tests, despite it not being used generally for PBC.I hope you do well on treatment, I am a lot better now than I was when I was at my worst I couldn't imagine ever feeling well but now I am so much better I hope you will feel better soon.Jane

[Clay6852 in reply to jane1964]

Thank you for your fast reply! I was shocked to read that PBC could be an overlapping disease of MPA. It’s really reassuring to hear that the drugs have helped with the liver. That’s what I’m hoping for! Are you now drug free? Did you have relapse?

[jane1964 in reply to Clay6852]

Hello I have been on azathioprine and hydroxychloroquine since diagnosis this has improved my symptoms about 90% I still have attacks when pain worsens but no where near as bad as before.Since being on azathioprine my liver function tests are all normal,and alkaline phosphatase which had been around 250 for 5 years has been consistently under 100.I'm delighted!

My rheumatologist says he might try reducing the azathioprine at some point and see if I replase but as far as I'm concerned I would be happy to stay on it if it's helped my liver I think the benefit definitely outweighs the risk for me.I 've gone from lying on the sofa in terrible pain feeling awful at its worst I felt like I was dying to bring able to do an hour's aerobics.I wouldn't have believed it possible.It's interesting you have such similar conditions I haven't heard of anyone else with this combination.I hope your treatment works well for you.Jane

[Clay6852 in reply to jane1964]

I am so inspired. By your story and progress! I too have spent many days lying in bed feeling like I’m dying. I’m so hoping to get through treatment and get my life back! I read an interesting article a couple of days ago where they had found 6 MPA patients with an overlap of PBC ! Thank you for sharing with me. Wishing you so much luck on your journey x

[Mooka]

Hi and a big welcome to this group. Unfortunately your story about taking a while to get diagnosed is not unusual. Do go to the Vasculitis uk website and read up on mpa. Vasculitis uk believes that knowledge is power over your illness. Do you have a doctor which knowledge of Vasculitis? If not you should consider looking for a specialist centre. Your current treatment is pretty standard. You may find you have to be pretty proactive in managing your health. Remembering your medication and booking appointments etc. It’s all pretty overwhelming for you at the moment but yes you can still live a normal life it will just need some adjustments and acceptance that you may have to pace yourself a bit. Do come back and ask your questions we are not doctors but we know what you are going through. Vasculitis uk also has a helpline should you need. Good luck x

[Cookyboy1 in reply to Mooka]

It took nearly 18 months for my diognosis, to late to stop it reaching my lungs, still trying to find out more about this desease but seems now I have joined vasculitus u/k I'm in tbe right place.

[Tbrz]

Hi, I’m so sorry to hear what you’ve been going through. I too was diagnosed in 2016 with GPA, after 2 years of misdiagnosis. unfortunately; this meant I had got to a fairly acute point and was very ill and have been left with a number of issues which heavily impacts my life now. I was treated at first with Cyclophosphamide and 120mg of prednisone and then a maintenance drug. Unfortunately this did not really control GPA and was still very ill, so we asked to be referred to Dr Jayne’s team at Addenbrookes, Cambridge. I was put on rituximab, still on every 6 months and I’m happy to say I am nearly in remission, although I am still on 10mg daily. I just wish I had been put on rituximab from the get go as I don’t think I would have such a long journey and might have been able to save my job, anyway we are where we are and I have to get on with things. I would recommend you ask to be referred to a centre of excellence for vasculitis as it is a complicated disease. I don’t know where you live but there are centres round the Uk so perhaps contact the vasculitis team for a recommendation of a centre nearest to you, so you can quickly get the best treatment and care. Good luck with everything.

[Clay6852 in reply to Tbrz]

Thank you for your reply. I will certainly be contacting my gp for a referral to a center of excellence. I’m pleased to hear you are in remission. Wishing you luck too

[Chris-Bromsgrove]

Hi, it sounds like you are now on the right sort of treatment for your MPA. You will probably be experiencing some of the unpleasant side effects of these drugs but they should knock you into remission. My kidneys took quite a bashing from the disease but they have repaired to some extent. Urine is now free of blood and protein and GFR is a bit below normal bounds but not causing me any problems. You will probably need to be on maintenance drugs such as Azathioprine for the rest of your life to keep it in remission but that's that's a small price to pay.

[Clay6852 in reply to Chris-Bromsgrove]

Hi Chris, yes steroids and cyclophosphamide are not pleasant in their effects. I’ve had two rounds but still have protein and blood in urine. I’m hoping the next round might slow it down. Yes they have mentioned Azathioprine for after. I’m presuming that’s tablets?

[Chris-Bromsgrove in reply to Clay6852]

I had small traces in my urine for about 18 months after treatment. The Azathioprine is in tablet form. I couldn't get on with it so they put me on another mild immunosupressant called Mycophenolate. The important thing is it sounds like they caught you in time before any serious damage was done.

[Bowlsjack]

I too have been diagnosed withMPO ANCA after more than 2years of of various diagnoses such as Ideopathic Pulmonary Fibrosis, and polymyalgia . After months of severe joint pains, exhaustion, pericarditis , loss of appetite and weight loss, the ANCA blood test was given and a kidney biopsy which confirmed MPO.

I’m on steroids and undergoing cyclophosphamide infusions. Each day is a challenge energy -wise but my bloods have improved and I hope to be ready for the Crown Green bowling season in a few months😊😊

[Clay6852 in reply to Bowlsjack]

Hello, sounds like such a familiar journey the more I read! I certainly know what you mean energy wise! I hope you smash the crown green bowling season!

[Bowlsjack in reply to Clay6852]

Thank you...hopefully the neuropathy in my feet will settle down and I have the strength to last a game!!

[Katiz]

Hi Clay. I was diagnosed with the same in May 2018 with no other symptoms than tiredness. I started the aggressive treatment the day after the biopsy, 3 days of 1000 mg pred, dropping to 60mg when I left hospital and cycles of cyclophosphamide. Now on 5mg pred and also azathioprine. Other than 2 weeks in hospital and another 2 weeks signed off after that, I have worked full time since. Not saying it is easy. I am a senior policy advisor and I certainly experienced pred head and chemo brain, and on certain days even thinking was hard, which did hinder my job on many occasions, but I worked in a very supportive environment. I was determined that I didn’t want to give in to the condition. I wouldn’t say I am the same as I was, but everything is under control, and I have had no relapses. so hang on in there. It will all work out.

[Clay6852 in reply to Katiz]

Fantastic to hear you are in remission and the disease is not taking control! Thank you for sharing! Yes just keep thinking by mid May Cyclo over and fingers crossed the tiredness will ease! It will certainly make working more manageable lol