14 year old son just diagnosed with MPA, little information on long term prognosis, does anyone know?

[DuncanC]

His lungs and kidneys have been affected, but still has 50% of his kidneys functional. Has been placed on cyto and pres. Any long-term studies out there on the young?

[PatriciaAnn]

Hello Duncan

MPA, like a number of the vasculitides, is commonest in middle aged people, affecting men and women. As with other vasculitides we are seeing more and more younger people being diagnosed. I do know of a paper on younger people but that is on Wegener's. However, there is a paper "Microscopic Polyangiitis" by Sharon A Chung and Philip Seo which you can view at:

ncbi.nlm.nih.gov/pmc/articl...

Have you discussed the problem of fertility with your son's doctors. The cyclophosphamide can cause problems for the future. One of the newer treatments for vasculitis is Rituximab which doesn't run the same risks as cyclophosphamide. This has been shown to be effective in some cases of MPA although it is not suitable for all patients.

Here is a link which you might also find helpful:

Microscopic Polyangiitis message boards

facebook.com/pages/Microsco...

Best wishes Patricia

[DuncanC in reply to PatriciaAnn]

That is really kind of you, Patricia. From what I have read so far it all seems so unpredictable. Assuming he moves into remission, do you know whether any future flare-ups are basically more of what we have already seen, or can other tissues/organs become involved?

All the best

Duncan

[John_Mills Volunteer]

If you let me know in what area you live I might be able to put you in contact with others with MPA. Also if we know where your son is being treated for his MPA that might help too Susan

[DuncanC]

Hi John and Susan

We live in Kent, near Sevenoaks, and my son is being treated at the Evelina Childrens' Hospital in St Thomas' in London. From what I have managed to find out, we seem to be in the right place, and the team there certainly seem well-informed. The treatment he is having seems to be that which (according to the papers/other on-line material) is the most effective at this stage.

Thanks!

Duncan

[PatriciaAnn]

Hi Duncan

I'm afraid I don't know the answer to the question you ask. In WG it is possible that a different area can be affected during a flare. In eight years I've had one major flare and that was restricted to the same area as when I first presented.

The prognosis in MPA depends on the severity of the disease and the amount of damage done to the organs when the disease was active. I do know that relapses can occur in MPA but they are less common than in some of the other vasculitides. Usually, in such cases, a temporary increase in treatment is required.

May I suggest that when you next take your son to see your consultant that you make a written note of all the things which are bothering you. During the consultation the physician should, if he's worth his salt, mention many of these things to you. If not don't be afraid to question him.

Best wishes Patricia

[Maggie20]

Hi Duncan,

I am 52 years of age and in the past was really healthy. . I've had microscopic polyangitis for 4 years now. it started suddenly with rash and joint pain, back ache and blood in my urine. Initially it affected my kidneys and I had large doses of prednisolone and cyclophosphamide,also plasma exchange transfusion to get the anca titres down. I progresses to azathioprine and steroids . I had two relapses once I had stopped the steroids and each time the azathioprine was increased and I was put on steroids for a while to settle all the inflammation down. I have been stable just on azathioprine( no steroids ) for the last 2 years and am very well. During a relapse, any organ ,as they are all have small blood vessels can be affected although it was my kidney function that was affected negatively each time. Kidneys of course are packed full of small blood vessels . Keep upbeat- make sure you take your son back to the consultant each time odd symptoms occur that way treatment can be given quickly to minimise organ and muscle damage. The GP's always look blank when it comes to this rare disorder ! Best wishes . Maggie20

[Rod-J]

Hi, I was 16 when first diagnosed with MPA. I had both kidney and lung involvement. I am now 45 with two children of my own. Due to a few flare ups of the disease in my late teens/early 20's I ended up with renal failure and at 25 had a transplant. Everything has been fine since. Actually the drugs for transplant are similar to those to prevent flares, which is very fortunate. I believe you should look for early signs of flares and get treatment quickly before any further vascular damage is done.

Rod