Newbie with possible vasculitis

[MotherGothel]

*waves* this may be a long story, but I'll try to keep it as short as possible!

Diagnosed with Autoimmune hepatitis in 2006 aged 15. Plodded along on azathioprine and prednisolone, with a few periods of non compliance and ups and downs with flare ups. Generally though in good health.

Around April 20 i started to get really bad hives. They started off abdomen/chest area but over the weeks that followed i was covered head to toe. GP prescribed Antihistamines which didn't work, so I had bloods taken in July where I had low platelets, raised ESR and CRP. GP panicked and told me to stop my medication as she believed it was a reaction to this. Referal was made to the hospital.

I had many blood tests done over the following weeks, platelets remained low and lots of other tests came back abnormal too. Ige came back REALLY high. They trialled me on steroids again, which raised the platelet count and stopped the hives. As soon as the dosage was decreased they came back. I was then referred to haematology who, over the phone, said it was my medication for the AIH, it couldn't possibly be anything else, and to continue having weekly bloods and she would speak to me again in 3 months!

Had to go back on Antihistamines to control the hives, ended up with 4 urine infections, blood and protein in my urine in dipstick test, plus visible blood in urine, headaches and migraines so bad I couldn't see and I was throwing up, joints became so painful I couldn't move them, and I was having constant pins and needles in my hands and in the end I lost feeling in my hands every morning for several weeks.

Liver doctor did a ultrasound and fibroscan, showing enlarged spleen, portal hypertension, cirrhosis of the liver and thickened gallbladder. They believe its because of the no medication for 7 months!

I was then referred to Birmingham for my AIH as my local doctor could no longer treat me as it had advanced so much without the medication. At this point the haematologist decided I now needed a bone marrow biopsy done before I was seen at Birmingham! That was done 4 weeks ago and I'm still awaiting the results.

Birmingham were brilliant and did a whole range of blood tests. They rang me a few days later to say that I had tested positive for p-ANCA and had a very strong positive for MPO antibody. They said as my kidney function looks ok, they believe it to be vasculitis.

Thats as much as I know for now! I'm being seen again next week (9th Feb) and don't know what to ask! I've been started on tacrolimus and prednisolone for my AIH, but they want to change the tacrolimus to mycophenolate instead as this is better for vasculitis they said. My hives, headaches, joint pain and nerve weakness has all eased since I was restarted on the prednisolone at 20mg.

Sorry its been so long! Any advice is greatly appreciated! I don't know what questions to ask when I go back to Birmingham and don't know what to expect from all this really!

[Chris-Bromsgrove]

Hi MotherGothel. Sorry to hear that diagnosis has been very drawn out but that is often the case with vasculitis. Are you seeing someone in Prof Harper's team at the QE? You haven't said what type of vasculitis you have other than it's ANCA associated vasculitis

[MotherGothel in reply to Chris-Bromsgrove]

I've not been told a type, just that they suspect vasculitis. I'm being seen for my AIH at the QE so am under the liver team at the moment. I'm not sure if they will refer me to someone else as well?

[Chris-Bromsgrove in reply to MotherGothel]

There is a specialist vasculitis clinic at the QE that I visit (pre pandemic). Could your specialist refer you to someone in Prof Harper's team? Diagnosis is normally confirmed by carrying out a biopsy on the affected areas. Some of your symptoms do point to possible vasculitis (MPA?)

[jackrussell in reply to Chris-Bromsgrove]

I agree with you there Chris ,I had extensive hives and inflammation ,and had biopsies twice ( 20 years apart ) and both times diagnosis the same , Vasculitis, I do wonder why there has been no biopsy on the rash. There may well be good reason, but perhaps you could ask .

[Mooka]

Hi MotherGotgel

Don’t worry about long story most of us are pushed from pillar to post before getting a diagnosis. Chris-Bromsgrove is right you need to be seen by a specialist team. If you are seeing someone new expect to go through all that has happened from start to finish. It’s a good idea to take any letters and results you may have with you. Usual treatment for ANCA vasculitis would be to hit it hard and to have High dose prednisone, weaned down quickly. Take a urine sample with you too. In the meantime have a look at vasculitis U.K. website as all the information you need is there. Keep away from Dr Google though. Good luck and let us know how you get on.

[metalback]

As others have said, Birmingham QE have a vasculitis clinic under Prof Lorraine Harper. You will probably need to go on cyclophoshamide and steroids to bring the Vasculitis under control. Then look at a maintenance drug. Don't be afraid to ask to see her or her team. Then ask for a treatment plan.

[MotherGothel in reply to metalback]

I've just had a letter through from my hep doctor, who said he has discussed my case with Lorraine Harper! The letter says "she was satisfied that in the absence of any renal lesion to suggest clinically active vasculitis we can continue with our usual treatment". My doctor said to up my prednisolone to 15mg for 2 weeks, then stay on 10mg, when I go back to clinic, he will change my medication from advagraf to mycophenolate or 6-MP.