HUVS

If you have Hypocomplementemic Urticarial Vasculitis Syndrome and you have tried many medications Methatrexate IM and/or PO, immurane, cytoxin, cellcept, Plaquinel, Dapsone without favorable results. Ask your doctor to try Rituxan. It took two doses 1 month apart and I have been spot free now for 4 years. But I did have HUVS for almost 15 years before they diagnosed it so there is quite a bit of damage including skin, bones, joints, and especially lungs. But I am doing well. Drive myself, shop, clean house. I am just very slow.

Hope this helps anyone. HUVS is so rare and unusual that there is just not a standard treatment yet. And I think it can be caused by many different things so it will be hard and take time. The Mayo clinic was able to confirm the spots internal and external even the soles of my feet and palms of my hands.

Write if you have any questions. I hope this medicine will work for some of you that are still having problems. It is very expensive, but there are grants and your doctor will know how to apply for them. I was able to get a grant for both of my does and only ended up paying about $100.00 American each dose.

I was so glad it was spots. It was easier to coordinate my wardrobe with spots rather than stripes. You have to laugh.

Thank you for letting me share my story and I sincerely hope it helps.

7 Replies

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  • Hi

    Good to hear you are doing so well.

    Our consultants have to apply for Rituximab at the moment in the UK but we are hoping soon we will be able to have it prescribed when needed.

    Many people have had favourable results when all else has failed.

    I am sure Richard will be in touch soon, he has HUV. You are indeed a rare breed.

    I only have MPA.

    You can co ordinate your wardrobe with anything now! And I am sure you will speed up gradually now you have it under control.

    Best wishes for the future

    Lynne

  • Hi. Its great to hear from a fellow HUVSer.

    I have HUVs. I am on many medications. They are

    Preds

    Mychophanelate

    Fexofenadine

    Plaquenil

    Ranitidine

    Rampril

    Inpedimide

    Savistatin

    Ivabravadine

    Antibiotic

    Aspirin

    Adcal 3

    Aledronic acid

    I also self medicate with intramuscular injections of 10mg piriton 2-4 times per day.

    My current treatments are ....human plasmatherisis every 2 months and 300mg omalizumab every 4 weeks.

    I have had in the past. Dapsone, ciclosporin, rutiximab, synthet8c plasmatherisis and 10mg per month of omalizumab.

    Unfortunately having HUVs means i am in the er 2-4 times per week but after years we are managing and our trying to live our lives as much as possible.

    I miss driving and.walking my dogs the most though still find pleasure in other things.

    As for my wardrobe i have to make sure my rosy cheeks dont clash with my clothes. :-)

    Sarah

  • Hi,

    Good to hear from you and that you've had success with Rituxan.

    I also have HUV. I guess I've been lucky in that I get into remission just using Mycophenolate (Cellcept) and steroids. As you say with HUV being so rare doctors are wary of trying new and unproven drugs with it. So it's useful to hear that Rituxan has been used to good effect.

    Fortunately for me the spots are long gone. Sadly my other half would say that my wardrobe hasn't improved though :)

    All the best,

    Richard.

  • Hi

    Many thanks for the advice and glad to hear that things have improved for you... My husband was diagnosed with HUVS 6 years by his kidney specialist, and luckily the spots were brought under control very quickly by steroids and cyclophosphamide.

    Just wondering how your lungs have been affected?

    Lungs are my husband's main problem, caused by the HUVS. He has developed a very rare lung disease called obliterative bronchilitis to go with the rare HUVS!! All this has disabled him and made him house bound.

    I look forward to hearing from you.....

  • Thank you for writing back. Yes my lungs have been majorly affected. I am on O2 24/7, Bi-Pap at night and when I nap. I am still very strong but have no stamina. I do somethings say wash dishes, or vacuum for just a few minutes and I have to sit and catch my breath. I take Spiriva once a day, Xopenex as often as I need it. If I am getting a cold or something else going on with lungs I can do the Xopenex once an hour. I take Dulera 2 times a day and ProAir is my rescue inhaler. The doctors where I live wanted to put me in a nursing home and I was still in my 50's. I wanted to see my grandchildren and see a few sights when my husband retires. So I found some doctors in Dallas who work with HUVS everyday and when I started seeing them 18 months ago my lung capacity was 500. Three weeks ago it was 1000. I attribute this to 5 mg of prednisone daily. It's a hard drug on the body, but it is keeping all the inflammation down and breathing is so much better.

    I have been very blessed that so far my kidney's have suffered too much. My biggest problem with kidneys is fluid retention so watching the diet and taking Torsemide 1 daily minimum, but can take up to 3 a day if I am swollen. I took Lasix for years and it worked well, but about 2 years ago it just did not cut it any more. Be careful and watch your potassium. Some folks have trouble with potassium on the Lasix and other have trouble on the Torsemide.

    I have also found if I can keep the air cold it is much easier to breath. Here in Texas we are already over 100 degrees daily and going outside is like opening the oven door and trying to take a breath. I just can not go outside in the heat. If I do have to the doctors have ordered me an oxygen regulator that goes up to 15 liters. Occasionally I do use that much. mostly I am at 4 liters O2 and like when vacuuming I will go up to 10 liters O2. Just be careful you can get CO2 poisoning so much O2 in going in that the CO2 can not get out. So I am very conscious about watching my regulator settings.

    Everyone is so different and the scaring and the damage done by the HUVS is so different for everyone. Mine are lungs also and my joints. They tell me that HUVS is in the family of Lupus and RA. They keep checking me for RA and it is still negative. It is just the scare tissue on the blood vessels that keeps the joints from getting all that they need. Just like the lungs and O2. So far we have not been able to do anything about the joints. So if anyone have any thoughts on joints I would be very interested. I am very lucky that my doctors really talk to me and we share ideas and try different things if they are reasonable.

    I have not heard of obliterative bronchilitis I will share that with my docs and see what they say. Down here in South Texas the doctors have not even heard about HUVS and they keep saying I have COPD. That is why I went to Dallas.

    Eyes is another problem. I went to an eye specialist and he took pictures of my eyes and said "See all those straight and smooth blood vessels? They are the healthy ones. Now look at those vessels that look like they are trying to write a name. Those crocked ones are the ones damaged from the HUVS. I do not really have any eye problems, but my glasses prescription changes quite often. Of course I would never want to admit that it was from old age. I use a lot of magnifying tools.

    I hope this helps. It was a pleasure hearing from you. God be with you and your husband. It sounds like you are a very good caregiver and I know that is not easy. Hang in there you are doing great.

    Holler back if you would like. Thank you

    Have a great day.

  • Bit late seeing this thread. Interesting that your prescription for glasses keeps changing - so does mine every 6 months or so. The preds caused cataracts in both eyes but that was dealt with. Current suggestion is its eyelid pressure on the eyeball so under the knife nest week. My lungs have recovered fortunately and attacks are now rare. For the record I'm on 10mg prednisone, 22.5mg methotrexate/weekly and Ruconest - not normally used for HUVS - but the nature of this condition is that what works for one does not necessarily work for others.

  • Hi there Texas195260

    This is a wild shot since this post is rather old.

    I'm a swedish woman with a HUV-diagnos. I've tried a lot of treatments incl. Prednisolon, Plaquenil, Dapson, Imurel, Methotrexat...without satisfaction.

    Then they gave me Xolair (omalizumab) six times and I got so much better. After the last shot I started to feel worse again. Though nu urticarias have disaeppered. Horray.

    Now they think a might have HUVS (which I suspected all the time) and they can't decide what to do next...

    How many shots did you get/for how long time? Do you have any other advise? Any information at all would be a help.

    Desperately seeking answers