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.

[Stevenelp]

I was also diagnosied with H-Pylori and IgG4 Sclerosing disease  on my arota.

Answer: H. Pylori (Helicobacter pylori - formerly known as Campylobacter pyloridis) is a gram-negative bacteria found in many people's stomachs. It was found in 1982 that H. pylori is associated with gastric ulcers and gastritis, and is a factor in developing duodenal ulcers and stomach cancer. However, 80%...

[sisterCarol]

My sister was diagnosed with IgG4 in 2011 in Ontario, Canada.  She taught Autistic children in Bancroft but retired in 2008 at age 60 due to fatigue, diverticulitis, sinusitis and IBS.  Spring 2009 in eastern Canada she got a bug byte on her leg that resulted in a tumor that was surgically removed.  Winter 2010 she visited Texas and Arizona.  By summer 2010 she was anaemic with a constant cough.  In 2011, bloated, tired and in pain, she saw doctors in Toronto and Oshawa who found masses growing around her kidneys, heart and lymph nodes and prescribed Hydromorphone for her cough, Lorazepam,  Aspirin, Dicetel for IBS, Bentylol for gastrointestinal spasms and 12 doses of Itraconazole in case of a fungal infection.  Biopsies don’t show lymphoma.  April 28, 2011 she was diagnosed with IgG4 related sclerosing disease and was prescribed 35 mg of Prednisone, gradually reduced to 3 mg.  Her masses stopped growing.  Fall 2011 she had 4 doses of Rituxan that yielded a low 20% shrinkage of masses.  
Winter 2012 her masses grew significantly and she had C Dificile 3 times.  She gets out of breath easily as a mass surrounds one of her lungs and doctors are concerned her heart may stop.  She is now on 60 mg of Prednisone, Nitrolingual Pump for her heart, plus drugs prescribed in 2011.  Her Haematologist sees no need for food allergy or intolerance testing.  Yet, I read 2 articles that say blood levels of IgG4 can increase due to food reactions like gluten intolerance which has similar symptoms.  Winter 2012 she was 90% gluten-free and had less pain.  Her IgG4 level has gone from 2.4 down to 1.4 (where normal is less than 0.8).  Is the reduction due to varying medications or eating less gluten?  If any of you other IgG4 sufferers try eating 100% gluten-free (no wheat, rye or barley) for 3 months, please let me know if your IgG4 level is reduced and by how much.  
March 30 she saw a mycoplasma specialist at St. Michael’s Hospital in Toronto, but he said her disease does not present like a mycoplasma infection so mycoplasma testing will not be done.  He contradicted Dr. Garth Nicolson’s belief about person-to-person spread of mycoplasma infections in Texas and high occurrence of mycoplasma infections in autistic children.  
Please give me any advice that may help my sister.

[dsmayer316]

I am a unique case actually. My situation began with swollen salivary glands, first one one side and then the other, then asthma, Next, my prostate/bladder became inflammed, then i had schleorising of my bile ducts, and inflammation in my upper small intestine. Next came awful hives, that would cover my entire body but only last 25-30 minutes and then vanish. All through this i have also had eosiniphilia. I have spent 2 years going through dozens of doctors. Finally went to Mass Gen and they found that my IGG4 level was almost 300, which was elevated. They felt strongly that I had IGG4 inflammatory disease, but some of these secondary symptoms didnt fit. Has anyone else had most of these symptoms? I was on Prednisone for 8 months and it cleared up everything, when i went off, my eosinphils and liver enzymes shot through the roof.  I got rituxan infusion in January of 2012, but it didnt really do much.  I have seen other drugs on here..such as Cellcept, Imuran...how are those working for everyone?

[sisterCarol]

Regarding small intestine inflammation, possibly you should read about Celiac disease:  http://www.celiac.ca
"Celiac disease is a medical condition in which the absorptive surface of the small intestine is damaged by a substance called gluten. This results in an inability of the body to absorb nutrients".  
Also try searching the Internet for gluten and IgG4.  Doctors tend not to test for gluten intollerance, celiac disease, wheat allergies, and other food allergies, yet I've read that delayed food reactions produce IgG4.
My sister's IgG4 count went down significantly when she ate less gluten.  
Good luck, Carol

[sisterCarol]

I expect this is my last posting in this web site.  Now that her IgG4 count is greatly reduced, my sister Mary was retested and rediagnosed with Rosai Dorfman disease and will be treated with chemotherapy drug Gleevec in pill form for at least 3 months starting mid June. Meanwhile she takes 30 mg of Prednisone.
I'm not in the health profession, but based on my sister's experience, I highly recommend that everybody with a diagnosis of IgG4 get their food intolerances tested and stop eating any intolerant foods.  Also, given that Prednisone is a powerful anti-inflammatory, I suggested Mary eat more anti-inflammatory foods and less inflammatory foods as this may reduce her need for Predisone.  She is:
Eating much less rice, bread, sugar, dairy, potatoes, pizza, pasta
and eating much more collards, carrots, swiss chard, garlic, kale, onions, spinach, sweet potato, almonds, hazelnuts, pecans, flaxseed, fish, beef, curry (for the turmeric)
She occasionally eats brown rice bread, pizza & pasta.  If she didn't have a gluten intolerance she'd eat whole wheat which is much less inflammatory than white bread, pizza & pasta.
I got information on Inflammation Factor for various foods from:  http://nutritiondata.self.com/facts/cereal-grains-and-pasta/5813/2  which enables you to look up individual foods, and from:
http://www.countdowntofitness.com/fitnessblog/2011/05/11/chronic-inflammation-and-the-dietary-connection

[Kay65]

I also have been diagnosed with igg 4 diease.  Does anyone know about long term effects? I have masses in lung, was at one time very sick, have been told it will probably cycle, would love any comments.