Help with rare diagnosis of IgG4 Sclerosing Disease??

[Christie1983]

I have recently been diagnosed with a rare autoimmune disorder called IgG4 Sclerosing Disease. I know very little about it and have had multiple surgeries and have seen MANY doctors regarding it. So far none of my doctors have seen anything like it and the only reason I reached this diagnosis was because one of my doctors reached out to a colleague at the MayoClinic in Rochester, MN who has 8 patients with this disease. I just want help understanding it and maybe to reach out and speak to anyone else that has this as well.

[KG100a]

Hi - I also have IGG4. Took a year to figure out why eye lid was swelling to the size of a golf ball. Had 3 sinus surgery's prior, some Asthma.
The eye swelling started 5 years ago. Have been on prednisone ever since. I get down to around 4mg and it slowly starts to come back some other place on my body. The latest is my arm due to a pulled muscle. Not much being done in this area. Thought I would post to show there are more of us with this problem.

[namaste4]

Hi there,
I was very recently diagnosed with this disease and I also have bronchiectasis, had two sinus surgeries, dry eye syndrome, and my salivary and parotid glands are affected.
Awaiting testing and tissue biopsy to confirm diagnosis and hopefully get off the steroids that have killed my bones, my blood sugar and my skin causing terrible bruising.
I only hope I will be successful getting off the steroids and get on a steroid sparing medication.
The last 5 years of my life have been a nightmare and now I fnally have a name to put to all that has gone wrong with my body. So frightening.
Cant believe how rare this is.

[Bobthebuildwr]

This sounds so much like my husband, he was diagnosed with IGG4 Fibrosing disease, we have been to Several doctors including Mayo. He has had the Rituxan treatment and is on prednisone but still is not feeling the best and we still have no answers he has been sick for almost a year and when we tell the dr. That this is not him, he also gained lots of weight.  He has a few good days but mostly bad... And not sure where to turn....very frustrating.

[sinusguyok]

In February of 2012 I was diagnosed with IgG4 RD while living in Los Angeles.  Lengthy pathology finally determined that what they had removed from my ethmoid sinus was not a tumor.  Since then I have connected with Dr. John Stone at Mass General, widely mentioned in these posts, and was part of a clinical trial he did with Rituximab.  My level was 35,000 at the time of the first infusion and zero after the second.  Yearly blood tests are monitoring it and when it gets too high we will do infusions again.  As nearly as we can tell, I have had this disease for about 40 years.  Ordinary testing does not really show my level of IgG4; it is in plasmablasts and requires specialized testing.  They now find that a significant number of people manifest in this way.  The only source I know of for this test is Mass General.  We now live in Oklahoma and have had blood drawn here and overnighted to Boston, and have also gone there for the draws.  I hope this info may help someone else who is trying to figure out what is going on in their body!
  

[RADHAC]

I have recently been diagnosed with igg4. I was told over the phone by my doctor but i judgment seen him yet to discuss this. I was getting sever pain in my kidney. Alot of testing showed it wasn't functioning. They were about to remove it but did abiopsy to find out its igg4. They put me on predisone A month ago before they got the result back. It helped but not completely. I also had parcartitis for 10 plus years (fluid in the linibg of the heart) they couldn't figure out what was causing it. Not sure if it could be related. Im a 32 year old mother i need to get treated with something that's going to help. They want to put me on rhimax, something like that.  I hope you get your problems figured out Shls.

[Shls]

I have been diagnosed with IgG4 Meningeal Dura and only 3 other cases to be known. It is very pain full my left side of my face hurts and head acks that will not quit.  I was given prednisone and the pain stop for about a week. I an deaf in one ear and going deaf in the other and yes it is from this IgG4. I was also told that this can cause blindness. I was on prednisone before and it did not work. I go to the specialist next week to see what other kind of drug they will give me.  I am very upbeat person and when one door closes another will open. I was wondering it the other meds have helped anyone else.