Last month I was diagnosed with PACNS, or Primary CNS Vasculitis. I've been taking daily prednisone and the plan was for monthly infusions of Cytoxan. When I visited my rheumatologist this month, he recommended switching to daily Azathioprine instead of the Cytoxan. I've only had one infusion of the Cytoxan so far and didn't have any major side effects. From what I've heard, Cytoxan is the most popular treatment plan.
Has anybody tried Azathioprine? What were your experiences with it?
I would want to know why he changed his mind.
I have cerebral vascultis, which is the same form of vasculitis. Fell ill 1994, diagnosed 1997. The consultant in my case opted for Azathioprine as the front-line treatment, because he was very concerned about Cyclophosphamide damaging my fertility (I was just 25).
Azathiorpine helped stablilise my disease, and I had a quasi remission 1998-2004, but then I had a huge relapse, and we struggled hugely to get it back under control. Eventually in 2012 I insisted on trying Cyclophosphamide. I think it turned things around for me.
I would recommend as strong a treatment as possible to whack things into submission, otherwise you are more likely to have a relapse in future.
Is your consultant experienced with treating this form of vasculitis? Does he/she maybe need to confer with other specialists for advice, for example the teams at Addenbrookes?
From what I've heard, he's one of the best Rheumos in the states and has published works on Vasculitis. I don't know if he's ever seen a case of PACNS though, as its so rare.
I worry that he's also worried about my fertility, as I'm only 23. When they first recommended the Cytoxan I told them that I didn't want kids anyways and that I was not concerned with the chances of infertility.
I'll definitely bring this up when I see him next week. I would also prefer the strongest treatment. I had three strokes in November. I'm not afraid of chemo, I'm far more afraid of future strokes.
Yes that's what my consultant thought too, and why he gave me only Aza, though I said "Just give me the treatment"
If you're sure you don't want kids and want to take the risk of infertility I think you should be able to get the strongest treatment, which should give you the best prognosis vasculitis-wise long term. It would be your choice,
Good luck!
P.S. Cyclo doesn't always lead to infertility. And sometimes there is time to freeze eggs in advance of treatment. But in a life threatening vasculitis it may be a choice of take life saving treatment or have more strokes and potentially die. Aza can also take up to 3 months after starting to be fully effective, whereas Cyclo works quicker.
It also seems a bit pointless to preserve fertility if you possibly wouldn't be able to physically deal with a pregnancy/child as a result of stroke damage. After all - in this day and age they freeze eggs or sperm before using chemo.
So, I met with my Rheumo and held my ground and insisted that I be given the Cytoxan. We agreed to do 2 more infusions of the Cytoxan, then go to Imuran. His reasoning is that my presentation is really good and all my doctors are very worried about the long term effects of Cytoxan.
Tonight (the night before my next infusion) he messaged me and said that they had a conference about my case and that after the infusion tomorrow, he insists that I switch to Imuran. His plan is for me to switch to Imuran the first week of February after only two months of Cytoxan. We're also tapering down my Prednisone, which I'm currently taking 30mg.
I am so frustrated because I thought that we had come to an understanding that I wanted the strongest treatment available. He seems really genuinely concerned about the long term dangerous of Cytoxan. But even Imuran has risks, most of which seem even more intense than the Cytoxan! And he says I'll be on the Imuran for 3-5 years.
Sorry to hear things didn't work out as planned. I suspect, unless you want to be really difficult, that you will have to go along with this medical opinion.
You will have had several chemo infusions however, so will have the extra power from them.
And you'd probably have ended up on Imuran afterwards anyway. After chemo infusions patients need to be on some other immunosuppressive drug long term, to maintain remission. Imuran is commonly used. Often for years, but occasionally for life, depending on how the patient does.
If it was me I would probably just accept the plan, and go along with it. But be alert for any sign that your original symptoms are worsening, or anything else concerning, that may suggest a relapse and need for urgent medical assessment and probably increased treatment again.
Also beware Imuran takes 3 months to be fully effective, so don't let the medics try to lower your steroids too quickly.
Good luck!
Hi Cunafish,
The problem with PACNS us that it's so rare ( 1 per million ) that there haven't been any clinical trials on the best treatment unlike ANCA associated Vasculitis. That said in the UK most cases of PACNS will be treated with Cyclophosamide to induce remission, as Viv says it needs to be beaten into remission!
Have you asked your Rheumatologist about the possibility of biologic drugs such as Rituximab if they don't want to use Cyclophosamide?
Was your diagnosis confirmed by biopsy?
I have not brought this up, but I will. For now, I will try to insist on the Cyclo unless they have a legitimate reason for switching me off it (other than fertility, which for me is not a concern.)
They chose not to do the brain biopsy, as the angiogram was extremely indicative of extremely widespread beading associated with PACNS.
I've been on azthioprine for about 3 years and have no complaint. I was prescribed it when I finished chemo and although I've had the problems associated with immune suppression I am being managed quite well. 175mg per day.
The concern in this case, and what happened to me, is that Aza is being used instead of chemo. Which may not be strong enough.
Six months of cyclophosphamide before I started on aza, I feel your apprehension.
Yeah, this is my exact concern as well. I want the most effective treatment possible. I understand that some women may take a different approach to preserve their fertility, and that's a total valid feeling I understand, but for me it's just not the priority.
I have CNS Vasculitis and have been on Aza 150mg for approx 1 year and we just realized that my daily migraines and hair loss are side effects of the drug. My doctor just lowered the dosage to 100mg and the headaches have decreased. Otherwise everything has been great.
I've received Retuxan Infusions which has worked out great for me. I have two more to go and that will complete my Infusions. Currently the Wegener's (GPA) is in a remission state with hardly any side effects. I'm off all other medications including the Prednisone.
After two more Infusions, 6 months apart now, they Doctors will only monitor me at that point.
Hope this helps
Hi, I was diagnosed with the same rare situation as you in 1995..I took cytoxan by tablet form, and took high dose steroids at the same time. I did this treatment for 9 months, and told the doctor I didn't want to do it anymore. They tapered me off the drugs slowly, and I have not required any treatment since. I also have lupus, and was diagnosed with the CNS vasculitis, lupus, and a very rare progressing eye disease called AMPPE... stands for acute multifocal placoid pigment epitheliopathy... long story short I was losing my vision by progressive scars over my retina. Seems all the conditions were all weird and rare autoimmune diseases, basically my body was attacking itself I guess! But the high dose steroids and the high dose of cytoxan for the nine months I believe knocked the disease out. It disabled my ovaries, and I never menstruated again either, but I already had two children so I wasn't concerned about the infertility side effects. I was 33. When I discussed treatment with my doctors and second opinion with the Mayo Clinic, they all agreed that cytoxan and prednisone at high doses were the strongest available treatment. At some point they recommended switching from cytoxan to Methotrexate infusions, but after tapering off everything I never returned to the doctor regarding.
So I would like to say to you to talk to your doctors about hitting the disease with the strongest available drugs on the market right now! Hit it hard and hit it fast, and have faith in a positive outcome!
Good luck, and best wishes to you in finding the best treatment plan available to you! My prayers are with you!
Starting Azathioprine
tapering off azathioprine 
Azathioprine and MPA
Whst to expect with azathioprine
