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.

[Whit_IgG4]

My dad was diagnosed with IgG4-Related Disease this year.  They found an inoperable mass in his brain that basically consumed his adrenal and pituitary glands and slithered up into the third ventricle of his brain, while also extending down the pituitary stalk and putting pressure on the optic nerve.  One of those infamous IgG4 pseudo-tumors.  The time we spent fearing inoperable brain cancer, made the IgG4-RD diagnosis a huge relief.  Other imaging showed inflammation and mild sclerosing in the kidneys, pancreas, and prostate.  In addition, his adrenal gland is permanently destroyed, doc says, and his pituitary is virtually non-functioning, and may or may not recover.  His body makes no testosterone or cortisol.

Diagnosis was made with imaging, IgG4 blood test, and biopsy of the kidney.  He went on high doses of prednisone for a couple of months and then Rituxin (4-6 infusions, don’t recall exactly).  His IgG4 levels have decreased, but are still high, and now he’s waiting for his follow-up with doc to find out if they will do imaging etc to check on his organs, or if he will do more Rituxin first.  He says he’s feeling better than he was, his prostate is behaving better, and his vision has improved a little.

Unfortunately, the prednisone caused Type-II diabetes that he’s managing through diet and Metformin.  He will need to take hydrocortisone and testosterone indefinitely.  He also has Barrett’s esophagus, and chronic sinus issues (he’s had 3 sinus surgeries).  

I can’t believe I didn’t find this place before.  I’ve searched a lot, and finally gave up and started an IgG4-RD group on Facebook when I thought I’d never find anything.  It is so cool to find other IgG4-RD people, even while I’m sorry any of us even have the need to look.  

I find the genetic discussion interesting and would love to learn more in that area.  The gluten talk caught my attention because 2 of my children have Celiac, my sister's gluten-intolerant, and my dad's mom had problems with wheat.  I'm going to go back and re-read everything (though I'll be skimming Nutribob's posts, as they were quite memorable.)

Nice to "meet" you all.

[bbastara]

I have been struggling with voice/breathing/eye/pancreas issues since 2012 but have not shown elevated IgG4 levels. This is a very interesting thread!

[lbrownlee]

WOW.. This is the most I have felt like someone understands since the 2.5 years they have been trying to figure it out..  Thanksgiving 2011 I got the flu..  Never got over the cough - Dec 20 my Husband took me to my primary..  They did an xray and my lungs were full on (what looked like cotton balls) he send me to a pulm.doc the next AM..  He looke at my CT and said get your affairs in order..  I think it is Lung cancer.. My world crashes.  The send me to a surgeon and they did a endoscopy then a needle biopsy and finally a lung biopsy..  NO CANCER... Then diagnosed as Histoplasmosis.  --Then Sarcoidoses-- Then IGG4--  I am on 20mg of steriods going down to 15mg--  All they say about what I do have- is that it is necrtizing granduloma..  aka Dead Tissue in my lung.  I have alot of pain around my lungs on the outside...  swelling then it will go down..    I also have saliva stones and type 2 diabeties and thyroid desease.. Can help but think all this is realated.  I am on a research panel at Vanderbuit her in Nash.. I would love to take charge of what I can..  I am 52 years old but I got a lot of life to live..... Any info would be appreciated.  

[humblebee1001]

I have been so sick for quite a while .Have had several surgeries o remove thyroid, salivary glands & perotid glands. I was put on Cellcept & Prednisone after being diagnosed with iGg4. I am happy tha I found this blog. I really feel the need to communicate & compare notes with other people experiencing this srtange condition.Doctors  are still trying to figue things out I had bad side effects from the cellcept. I am on Imuron and Predisone now. It will be good to visit with people who are actually experiencing what I am. Notjust dr. God be with us.

[Qwerty1594]

BreanaNadal,  I am in the oc area and struggling to find help from my doctors I've asked them for referrals to either cedars or UCLA  but it falls on deaf ears. I am now reaching out to dr stone in Boston to see if he will accept my case. Please share if you find any good local resources in the la area!!

[amymarcinko]

Hi, I have had the lung problems and the salivary problems.  now it has attacked my left orbital in my eye.  that's a tough one.  have you had that problem?  the methotrexate helps my other symptoms but the eye is awful.  I cannot even explain the symptoms buy you don't see out of it; but then you do.  it's creepy.  do you take something for the eye?