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Help with rare diagnosis of IgG4 Sclerosing Disease??
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.
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I do not particularly know someone who has the same form as you do, but i have knowledge of it. It is a auto immune pancreatic disease which means your body is attacking the pancreas, and it can also be systematic, which means it can attack ofter parts of the body. It is a genetic disorder, which means that it comes form your genes. Istead of making the antbody  IgG, you make IGg4. It attacks your pancreas over andover again, sticking many times, which may cause tumors. Luckily, you  do not have one of the worse, (but more common) types.
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Almost all of this statement is incorrect. igg4 does not attack , the symptom of the disease are fibrosis scaring and swelling. At this time they do not know what causes it. The area infected by it will most likely show and excess of serum igg4 antibodies, The only way to prove that you have it is a biopsy of the infected organ or tissue;  If it shows a certain pattern you probably have it,
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I am a patient at the Mayo in Rochester and have an IgG4 Sclerosing disease diagnosis.  Currently the disease is not affecting or "attacking' my pancreas.  It is, however, affecting my submandibular salivary glands and my pulmonary function, which is how I ended up at the Mayo's Pulmonary and Critical Care unit.  I've been on a lengthy (18 month) prednisone treatment, just tapered down to 0 a few days ago, and unfortunately my symptoms are recurring.  Would be curious what symptoms you're experiencing.
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Has there been mention of trying Imuran?  It's being used now to treat autoimmune pancreatitis which is closely related to what you've got, also the elevated IgG4. My AIP wasn't responding to repeated treatments of prednisone; symptoms returned each time shortly after the prednisone weaned down to 0. Have been doing great on the Imuran x 15 mths.
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I too have an IgG4 Sclerosing Cholangitis diagnosis. It's also affecting my lower intestinal ileal pouch where my colon was removed previously for Ulcerative Colitis. It was a mystery for years. I'd respond amazingly to steroids but get sicker and sicker when we tapered. Diagnosis was just made recently at Cleveland Clinic.

My advice Christie, is to visit Mayo or google and contact Dr John Stone at Massachusetts General via phone or email, tell him about your symptoms and diagnosis. He may want to look at any biopsises or see you in person. He's one of a handful of doctors observing and publishing on IgG4-Related Sclerosing Disease.

As TD2464 mentioned, it can manifest in places outside of the panceras/liver/bile ducts and researches are in the learning phase right now, waiting to see where it pops up next. The more of a common problem it becomes the better hope of real treatment down the road.

Hang in there!
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I have been on prednisone and methotrexate since September, I was weaned off from it in November but then I ended up getting really sick and spending 4 days in the hospital so they put me back on it. I was on 60mgs for awhile but now I'm on 40mgs. The side effects have been horrendous. I started the Rituxan infusion treatment the week before Christmas and I have had 2 infusions so far. I have a doctors appt in March to see if it is doing any good.The symptoms I have experienced have been intense facial and head pain, along with masses which is the inflammation. I am on the pain patch to control the pain to a tolerable level. My doctor has been in contact with a doctor in the MayoClinic in MN, the doctor has written reports on this disease and is treating 8 patients with it, he is also using the rituxan treatment. Very recently I have been experiencing new symptoms that worry me, I have been getting pressure in my head...then the sound gets very quiet, like things are far away, and then things get blurry and I have to either sit down or lean against something. Will be mentioning it to my doctor when I go to see him. My disease has manifested in my maxillary region as well as orbital and sinus region so far.
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Now here we have to ask an important question.  Is this truly a genetic disorder, meaning is it from your genes, or is it because some infective agent such as a mycoplasma is inserting into your DNA to cause the expression of IgG4?  Seems to me this is a question that you should be putting to your doctors, who probably won't know right offhand, but it may get them thinking, and looking in what I feel will be the right direction.
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I have just been diagnosed with IgG4 sclerosing cholangitis two weeks ago.
I live in australia,not many cases here.  My dr has 7 patients he has collected of 10yrs.  I started on 50 mg per day cortisone and have had an improvement in blood test already.  Dr hasn't given me much info.  Some good websites would be of great help.  The cortisone has sent me a bit manic, can't sleep.  Its take it of have a dead liver so no choice.  The secondary version is better than primary version, possible liver transplant with that.  That was my first diagnosis, which sent me into a spin.  I.m not sure yet what effect it will have on my life.  My husband has had gastric cancer and has no stomach so I am the breadwinner.  I have an appointment with Dr in a couple of weeks so will have lots of questions for him.  I wish you all well.  I have been searching every night for a forum, so this is great.
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Now that is interesting.  You see mycoplasmas have a defense mechanism, those that are intracellular can force that cell to produce a hormone that is is in the placenta, Human Chorionic Gonadotropin (hCG).  Said hormone's normal job is to protect the fetus from the mother's immune system.  Immune cells that touch that hormone self destruct.  Cortisone turns that hormone off and would allow your immune system to get at those infected cells.  Most doctors however will refuse to believe that this is a mycoplasmal infection.  Maybe someday they'll get their acts together.  Free fatty acids would have the same effect as cortisone, however if your liver, gallbladder and/or pancreas are infected one would need bile tablets or capsules to break down oils into free fatty acids.  Maybe your doctor will be a bit more receptive to idea that your dealing with a mycoplasma than those in the US.  Treatment of mycoplasmas is very tricky, they have more than this one defense.
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I was diagnosed in December 2010 with IgG4 RSD. It affects one side of my throat (removed one tonsil in November and biopsied it for the diagnosis) and the lymph nodes on that side of my neck.  I also suspect that my sinuses are involved.  Prednisone gave me immediate relief.  They are now tapering me off Prednisone - currently at 8mg/day.  They started me on a generic form of Cellcept in December and now doubled the dosage as I was tolerating it OK.  Will keep tapering off the prednisone at the rate of 1 mg every couple of weeks if I can.  I see the Dr. again in May. I am only in my mid fifties and now have Osteoporsis - so Prednisone is really not good for me but there is no choice for now.

I am seeing a Dr. at the University of MN Rheumatology Clinic who has treated IgG4 patients previously, just not in the area where I am affected. He is very sharp and seems on top of this disease. He has a good relationship with the Mayo Clinic in Rochester and the doctors there who have treated IgG4.

I wonder if there are others out here with the problem in the neck/sinus/throat area?  How are you doing? I can't find much at all on the Web about IgG4 in that area of the body.

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There are, quite frankly, many who suffer from the same.  However they all go through a bunch of misdiagnosis.  The diagnosis are widely varied, all have the same underlying cause, which I have put forth above.  If you all don't have mycoplasmas, then I don't.  I've been packing mine since 1968.  If you research predisone, you'll see why it is effective, as is cortisone above.  One being a natural hormone, the other a man altered hormone, so the drug companies can charge an arm and a leg for it, hormone.  I'd suggest you ask your doctor to look a little deeper into that biopsy.  I guess it takes a lot of education to become as blind as some of them are, I only have half as much, thankfully.  Out of curiosity, which side of your throat is affected?  I'm going to guess your left.
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It isn't the mycoplasmas causing the overexpression  of IgG4.  Not directly.  They are what causes the sclerosing, making themselves comfy in their new home.  The destruction of the IgG4 that takes place as a result of the infected cells producing the placental hormone is what causes the overexpression.  Your body makes more and more to try to compensate for the loss.  Some tumors have the same defense mechanism.  Why?  Because in reality, they are in many cases, also mycoplasma infections.
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It's the right side of my throat that is affected.  They have biopsied and tested blood serum and are quite confident in their diagnosis.  Thank you for sharing your insights.
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Well I had a 50/50 chance there.  The right side of the lymphatic system is separate from the rest, just the upper part of your body from the waist up.
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Here's some interesting research I found on IgG4:  Because IgG4 is predominantly expressed under conditions of chronic antigen exposure, the biological relevance of this exchange of half-molecules is that it generates antibodies that are unable to form large immune complexes and therefore have a low potential for inducing immune inflammation. In contrast to monovalent immunoglobulin fragments, these scrambled immunoglobulins have a normal half-life. The significance of the ensuing bispecificity needs further evaluation, because this will be relevant only in situations where high IgG4 responses are found to two unrelated antigens that happen to be present in the body at the same time and place. In this context the significance of IgG4 autoreactivity might have to be re-evaluated. The main function of IgG4, however, is presumably to interfere with immune inflammation induced by complement-fixing antibodies, or, in the case of helminth infection or allergy, by IgE antibodies.
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Now, the last line of the above got me thinking, and that got me researching.  It seems that IgG4 is immunosuppressive.  I found some research that shows worms, that's what helminths are, are able to produce an immunomodulating substance that causes the overexpression of IgG4.  Why?  Because without inflammation caused by the more active immunoglobulins, one doesn't even know they're in there!  That is what I said in the beginning, there's something inside of your cells causing the sclerosing, and they don't want to be discovered.  They're know as mycoplasmas, and otherwise known as Stealth Pathogens, which is the title of a book for those interested.  Here's the research wherein I found the info on helminths and the fact that IgG4 is immunosuppressive:  http://www.jimmunol.org/content/174/8/4718.full
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So, I would like to know how to diagnose this disease? My son is very ill. We have been on a hunt for about six to seven years now and he is only getting worse. When he was 16 he broke his back playing football for our high school. Then he has had three to four compression fractures in his spine within two years of that doing just light stuff. He has never got out of pain with his back since and also has had two surgeries on his back for the break. We wondered why he still lived in pain day in and day out. Before his back break he had sinus problems with two sinus surgeries. He goes to a pain Dr for his pain, his pain is not only in his back but now has moved to his joints, and all over his body. We then thought he had cyclic cushings disease and have been out to the NIH in Maryland for testing. He goes in cycles where he gains water weight like crazy. He has now gained over sixty lbs since March when he started a cycle. He has also formed like blisters on his lower back that turn into ulcers and bleed. Where that has come and gone his tissue under his skin on his flanks and under his periumbilical area is sclerosing. He has hardened tissue. He is very miserable! And no Dr. can explain why? His jaw by his ears really hurt him especially in the joint of the jaw. He complains that his ears hurt, ring and then sound will sound far away and then his sight will be affected too. He also has been having horrific headaches. He also cannot urinate very well. Im assuming its the prostate that is enlarged? We did find that his Igg4 was elevated at 167 a year and a half ago and now its 264. This and his cortisol is really the only labs that have been off. I have a Dr. trying to contact Dr. Stone at Mass General, but no answers back yet? He recently had a CT scan done on his abdomen and it showed fatty inflatration in the liver and in pancreas. I wonder is it fatty inflatration or is it sclerosing and they cant identify? His gallbladder was also collapsed. Anyone got any of these symptoms with your Igg4? How do we get it diagnosed. He is just suffering. He has had to have a pain pump placed inside of him because his pain is so intense. Thanks for your input....
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I forgot to add that he is pretty much bed bound. I can't even explain just how sick he is....
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Gees, if I could remember everything. He has also had tissue biopsies from his hardened tissue and they find eosinophils in them. He is also having them found in his digestive system. He has had bleeding ulcers in his duodenum. His pain Dr. Says he acts septic?
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Eosinophil granulocytes, usually called eosinophils or eosinophiles (or, less commonly, acidophils), are white blood cells that are one of the immune system components responsible for combating multicellular parasites and certain infections in vertebrates.

It's like I said above.  Your pain doctor is right, he is septic.  It's probably little vermin known as mycoplasmas.  The hard part is finding a physician who even knows what the word means!  I don't know where you're located.  But if you search for Dr. Garth Nicholson, you'll find his website, immed(dot)org. There he would be tested for mycoplasmas.

Some of the symptoms he is showing also sounds like Lyme disease is a possibility.  Spirochetes are known to form the blisters you mentioned.  Sounds weird but you see, the Lyme spirochete can change to a mycoplasmal form.  I went through the painful jaw/ear thing many years ago.  I've had this stuff since 1968 when the AF came to our little town in Ohio and gave us immunizations for typhoid.  I'm sure you can find a Lyme Literate Physician in your area for testing.  Best testing is done by IGENEX>
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Thanks for your input, I will check it out. We are in Utah and have been to the U Of U medical center at the Infectious Disease clinic and so far  nothing has showed up. He has tested positive to an autoimmune disease, so it points more towards the Igg4. His Dr.s have come to a conclusion that he does have something systemic. What are mycoplasmas? I will go and research it. He has been tested for all parasites and none are found....? So what are you? Do you just do a lot of research? Or are you a naturalist? Im so sick of getting the answers of I dont know what to do for him...he also does this neurological thing where it looks like he is seizuring but he is not. We have had him to neurologist but they cant figure it out either.
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My husband was diagnosed with IgG4 Sclerosing Cholangitis/Pancreatis last April at the Lahey Clinic in Burlington MA.  He had been losing weight off and on for about 5 years which was always passed off by doctors as age related.  However, about a year ago his salivary glands became very enlarged and he was referred to an ENT who did a biopsy for cancer and found none.  This doctor suspected some type of autoimmune disease.  We then began an endless round of doctors visits and tests, i.e., bone marrow biopsy, colonoscopy, endoscopy, PT scans, etc. which turned up nothing definite.  At the end, our cancer doctor suggested a wait and see attitude.  In the meantime my husband had now dropped 40 pounds and had a sed rate of 130.  I then decided we had to take the bull by the horns and go to the Lahey.  We spent a month there and after several tests and a liver biopsy, he was diagnosed with IgG4 SC & P.  We live in Florida but are originally from MA, hence our knowledge of the Lahey Clinic. He was referred to a local gastroenterologist for treatment with prednisone 40mg a day and Pancreaze 4200 units 3 X a day.  He has responded very well to treatment so far, however he has developed diabetes from the Prednisone and is now on insulin shots every day.  As everyone here has stated, we don't know what the future will bring or how it will be when they begin to wean him off of the steroids in another month.  Will try to keep you posted.
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Last December I had a biopsy done in my sphenoid sinus as a mass was found on CT/MRI.  Surprisingly it came back as IgG4 Sclerosing Disease.  Which the doctors said they have never seen in the sinus cavity before.  I was referred to the rhuematology dept of the hospital I'm treating at, but no treatment was prescribed at that time.  Next week I am going for a PET scan to see if other areas are affected as well.  At this time I have have no real symptoms or problems.  So I will be interested to see what other areas are affected especially after reading your post
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My father is very sick for more than 7 years. He has been treated for lymphoma with no success...
He is in bad abdominal pain for years. He can not breath..
Now we think he has IgG4. He has problems both in lungs and pancreas. Maybe also IgG4-cholangitis.
We are not sure what is the best treatment when both lungs and pancreas involved. Because this is a new entity, we must make a research by ourselves...
Will be glad to hear from people with lungs involment.
Thanks,
I was very happy to find this forum.

  
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Hello I too have been diagnosed with IgG4 disease, I found out about 1 and half weeks ago, which was scary because no one knew what it was they had to send out the biopsy to UCLA for diagnosis any way I am now on prednisone 60 mg.
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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%...
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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.
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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?
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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
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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
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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.
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Hi........ i've had problems without knowing, since i was 16 years old.. now I am 57. I  have had surgeries to many to count of things growing without explanations. Until a month ago did they discover igg4 disease. My life, I've sufferes with unthinkable rashes and sickness........when I wanted a break from all this, i'll take predisone for a while to feel human and once off back to being sick and itchy and things growing inside. I've had mass removed from lung involving taking most of the lung. Jus got off a round of steroids enjoyed the relief, but things are returning back to my old self...but its ok too.
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My 9 yr old son was diagnosed 12/14/12 with Igg4 related syndrome!  This has been a long drawn out battle, years of high fever, rash, pain, joint swelling, abdominal pain,   hundreds of lab draws...variety of imaging scans.The problem i am finding is that Igg4 is a relatively new diagnosis, with not alot of literature regarding it.  According to the doc who just diagnosed my son....there are no reported cases yet of Igg4 related syndrome in a pediatric patient, because of this fact he has contact Dr John Stone out of Mass General Hospital! Dr Stone is the leading expert in this area, publishing papers to the New England Journal of medicine regarding this illness. Dr Stone has asked to follow my sons case, that he would like to examine him and overlook his labs, possibly order more ( Poor kid is sick of being picked and prodded) that the fact that there is now a pediatric case, he has hopes of learning some valuable information through my son regarding this disorder.  I wish all of you well.
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Some of your sons symptoms sound very similar to my sons, but not to the extent your son seems to have, my son is 9 At the age of 5 he contracted lyme disease. Lyme disease is a very nasty infection....that many docs are still clueless about!  Lyme disease is caused by a spiral-shaped bacteria (spirochete)  Some strains they are learning are resistant to some antibiotics, so you can be treated, thinking you are getting better, but what actually transpires is the disease goes into silent mode, the disease seems to recycle every 4 weeks, and the spirochete love low oxygen environments ( Like joints )  When the spirochete enters into a joint it is called disseminated Lyme disease, which cause's  swelling, pain and stiffness in the joints. Has your son ever had a ELISA or the WESTERN BLOT  test performed?  If not i would recommend asking his primary to perform it, but realize....30% of positive people will test as a false negative. and the reason is because new studies are showing that the spirochete can make the host ( your son and my son )Immunio- suppressed, which means their bodies are not producing any antibodies.....because their system is compromised and cannot fight it!  I am not sure about what labs have been done on your son, but some tests that came back positive with my son are ESR level of 100 CRP level of 15.3 Moderate titer antinuclear Antibody of 1:160 and 1:320 Speckled pattern. Igg4 with values of 113.3 followed by repeat igg4 5 days after first draw with value of 125.3 .  I have had to fight tooth and nail...meet with doc after doc, to get to this point.  One doc was horrible the initial doc who diagnosed his lyme disease, mistreated him....put him on a 5 day course of antibiotics.....which sent the lyme into silent mode, and it wound up in his joints! Also 10% of people who are infected by Lyme....will go on to develop an Autoimmune condition. The Lyme disease had almost a year to damage his body before finally a doc truly listened to what i was saying, and looked over my logs. I would suggest you keep logs as this doc said my logs really provided him with detailed information and helped him to choose where to start testing for other issues! I kept logs of...Fever, pain , diet, sleep pattern, bathroom habits, fatigue, rash's ( I took pics of the rash ) Its hard for them to dispute facts written in black and white!  I had been keeping these logs in a regular notebook and learn when the cat spilled my coffee all over them that perhaps i needed to discover a new outlet for my logs....which sent me in facebook's direction, I created a private group ...which i titled " Observations"  and i  go there daily and list everything, it also gave me the option to be able to place photos of any rashes into the group as well. I was able to link his dr to the group and he gets to view all my info at his availability, and he is able to leave me feed back or ask questions as well.  I know all to well the frustration , anger, and sadness that goes with all this, I will include the link where i am getting  "Recent Lyme disease information" They have a symptom check list...which you can view to see how many he has.  It is called ILADS.org. Good luck to you and your son....I hope you find answers soon.....Not knowing is always so hard  {{{Hugs to you}}}}
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Hi...my son who's 14 has been diagnosed with the Igg4-RSD on 26/12/12. About 18months ago he started to have problems to eat...the food got stuck in his oesophagus. After trying all kind of treatments, without any improvement, and loosing 18kg (+/-40pounds) a radio showed a "masse" compressing his oesophagus from the outside. Since the 21/11/12 my son has a nasogastric tube to be nourished. Only liquids pass. A biopsie of the mass led to diagnose the disease. He was hospitalised for 1 month. Now my son is at home, he even goes to scool with his nasogastric tube/food. Since 1 month my son is taking 32mg Medrol (cortisone) a day, They hope this treatment it will make the tumor shrink. For the moment there's no improvement. Does somebody know a similar case?
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I was diagnosed with IgG4 just over 6 weeks ago. Lymphnodes in my chest are inflamed, causing all sorts of issues with breathing and blood flow. In addition, it appears Anemia is another side effect of the IgG4.  I've been on Predisone for 5 weeks now, and the mass in my chest as actually gotten larger.  

I'm trying to get in with Dr. Stone, willing to travel from Utah to see him, but I'm being stonewalled by the receptionist saying he's booked to July. If you have any additional contact information for Dr. Stone you could pass on like an email address it would be greatly appreciated.
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I was diagnosed with AIP a year ago and concurrently had high thyroid antibodies.  I was also experiencing dry mouth and dark urine (temporarily).  After rapidly losing 15 lbs, I was finally diagnosed and treated with high doses of prednisone (5 months) which finally put me in remission (although I still feel get varying sensations in my pancreas).  My father has had similar problems but also has RA and went through a lung cancer scare (it was inflammatory pneumonia) and has had kidney problems periodically.  He also suffers from congestive heart failure.  My research leads me to the conclusion that we both have igG4 systemmic disease (I had high IgG4 levels during my flare; they have normalized for now as have my thyroid antibodies although I do have thyroid nodules).  He remains undiagnosed.  I am anticipating that I may need future medical care but not sure who to see.  A rheumatologist?  I see that Mass General is leading the charge, but I live in DC, not Boston.  I don't want to go from specialist (had GI doc for AIP and endocrinologist for thyroid) to specialist trying to treat each organ but would prefer to have someone look at this systemically.  I live in DC area.  
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Hello. For the past six months I have had bilateral submandibular swelling. At times my tongue has gotten a bit thick and the akin under the tongue swollen. It has completely altered my life and it is very difficult to eat, talk and sometimes affects my breathing.

A couple months before this I had problems with both legs feeling very tight, painful and pressurized. It almost felt like if you took a pin you could pop them to relieve the pressure.

Last month both legs broke out into red what I thought were massive hives. The Drs said possibly livedo. They went away in about two hrs. I took photos so that was good.

I have seen over 15 Drs and no diagnosis. ENT's first thought sjogrens then that came back negative. Rheumatologist thinks igg4 and just took some blood for it.

My question is how do you get a definitive diagnosis for igg4? Is it blood or do they need to do some kind of biopsy?

Has anyone else had submandibular or tongue swelling?

I have had over 200 blood tests. I am coming up negative for everything. My ANA is negative and my sed rate is normal. My c3 levels are low. The Drs keep checking C3 and C4.

I am hoping once I get a diagnosis I may start getting some relief.

Any help would be appreciated. Thank you
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I hope everyone is still reading this post!! I am 44 years old and have just been DX with IGg4-RSD. I have read this entire column and have not found anyone who has a similar case to mine. About 2 years ago I developed a pseudo tumor behind my L eye. Results from a biopsy showed that I had the IGg4 in the inflammation. I was put on 80mcg of Prednisone and tapered off of it after about 8 months. I put on over 130lbs. Once I was off of the steroid, the inflammation came back. This time they tried Methotextrate and that only managed it. I am in Michigan so I was referred to the Kresge Eye Institute where my doctor is somewhat familiar with IGg4, but has been following clos procedures to Dr. Stone from the Mayo clinic who seems to have the most experience. I will be going for IV infusions of Ritximaub (Rituxan) once a week for 8 weeks and then monthly afterwards. Scary!!! Anyways, I have been having other issues (maybe symptoms) that I was wondering if anyone else could share with me and compare.
The fat that I have accumulated is very thick. My body aches in pain and there are parts of my body that will tremble at any given time. Of course there is ALWAYS localized pain in my head from the pseudo tumor forcing my eyeball into my brain, and then theres the headaches. Somedays I can't even get out of bed. I have inflammation in other parts of my body, such as in between my knuckles and my ankles are so swollen. Does anyone else have anything like this?
  Sadly, I have other illnesses besides this; I also am type 2 diabetic, I now have sleep apnea from the steroids, I have high blood pressure, and I have been diagnosed with hypothyroidism.....but I thought I remember reading somewhere that some of the symptoms from IGg4-RSD are the same as hypothryoidism.
  I have not been able to work because of all the pain and not feeling well so I applied for social security but because my doctors (Primary, Endo, Rheumo, Pulmonary) aren't familiar with it, they can't really say if I'm able to work or not. At least thats what I'm thinking. My primary just throws his hands up in the air and says "I don't know what to do for you".
  Please, if anyone else has any suggestions or can tell me about their experience with this disease, I would love to learn more. I pray for all of us to recover soon <3
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Steve Hampton Va
I too have this dreaded IGG4 disease, I'm currently being treated by the VA.
I'm on megadoses of pednisone.  This turned me into a diabetic.  Now I have to take about 1300 mg of Metformine.  I have a tumor about 12 inches long behind my right lung down my spine.  So far both biopsies were negative for cancer.  I suffer with unbearable pain.  So I consistantely take pain meds.
Just had another CAT scan the other day.  We'll see what happens.  My Sympathey to all who suffer with this illness.
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Steve
mjzolt - my sympathies.  I to suffer every day with the IGG4 illness.   I already had anxiety and depression the steroids make it worse.  Sometimes I stay up all night between the rise in my blood sugar and the steroids.  The tumor I have is about 12 inches long down my spine.  Causes mega pain throughout my back.  So I finally gave in to the pain meds.  Had another CAT scan the other day.  We'll see?  Stay strong my friend
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Some of these doctors have no bedside manner and can't emotionally handle a patient who knows their body or asks to many intelligent questions.  Beware of the god syndrome. Yes I too have IgG4 and its been a long haul.  I've had 3 sinus surgeries for fibrious dysplasia in the sinus cavity.  I have had 5 supraclavicular biopsies done.  My blood work showed increased CRP, SED, platelets, IgG4.  The only thing that really helps me is predisone.  I am doing the rituxan and I can't say I really feel any different.  I've gained 20 pounds in 3 months and have several nodules on my thyroid that I can't seem to get anyone to check.  My stomach swells like a hard ball but again CT and ultrasounds show to be negative.  I would very much like to find out the cause of my abdominal distention but when I tell my doctors I have never looked like this before this is not normal for me.  I am told "get off your but and move you have fibromyalgia".  It seems to me doctors even the so called specialist are so caught up in their own importance to listen to the patient.  Beware of doctors who just want you to fit in a box or label you with a disease you don't have.  When a doctor says to you I see thousands of people like you a year be careful because they are not seeing YOU. Three years previous to my IgG4 I was told I had Castlemans Disease, now its IgG4 what will it be 3 more years down the road?  My guess cancer that has been missed or confused with a rare autoimmune disease.  My wish would be that they could come up with treatments that do more good than harm.
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When I asked  my MD what the determining factor in the diagnosis of IgG4 he said when they look at the lymphnode there is a scarring seen.  It is the scarring that indicates IgG4.  I don't know if this information helps you.  If you have a lymphnode removed pathology would need to look for that.
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Is your sister getting any better?

My mom also recently diagnosis with igg4 rd. I live in Ontario but no dr can referred her to any specialist here.. her eye surgeron ( she has tumor behind her eyes and after removed the tumor, found out it is igg4 ). family dr, oncology... no one can give me a name to follow up..

Which dr do you see?

thanks
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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.
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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.
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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!
  
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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.
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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.
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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.
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51 year old male, newly dx with igg4-rd found because of issues with my kidneys.

Had a biopsy because was having high protein and blood in urine, also experienced vasculitis, sinus issues and over the years had a few lymph nodes removed because if swelling (I was 29 years old when lymph nodes were removed and biopsed but igg4 stain was not defined then so the pathology report showed nothing).

I was (7 years ago) dx with SLE lupus and for a few years was on prednisone for that. But after a few good years of being off of it , symptoms came back but not the positive Ana.

I am on 60 mg prednisone and only a month into treatment so not sure of the next steps. I am convinced that I have had this issue since childhood and am just happy to call it something.

I have no pain, I don't walk around with a burden, I just want to treat and move on! The only struggle I have is keeping weight off but with a strong will and good strong diet I have managed to only gain about 7 pounds so far.  
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I am in the process of being diagnosed With IGg4.  We are waiting for the results from my lymph node biopsy.  Thy doctors at the Lahey Clinic in Burlington MA have been wonderful.  I recently did a biofeedback back session in my hometown to find out what my food intolerances are and I have been slacking off on that.  Milk and sugar are high on the list.  After reading your blog I am going to start taking my food intake much more seriously.  Thank you for posting!
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My husband is suspected of having IGg4 and has been recomended to go to UCLA as well after (6) previous doctors have been unable to help him. Would you mind letting me know who you saw when you went there? I am just trying to find someong who can help him. Thank you so much!
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I was recently diagnosed with this disease in November.  The leading specialist in the world is Dr. Stone out of Boston, Mass.  I get my treatment at Johns' Hopkins Hospital in Baltimore.
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I've had the orbital problems associated with the disease, and that has been the most incapacitating.  besides being on methotrexate, my doc has suggested something like maybe restasis for the eyes.  but I also get the formation of this film across my eyes which totally makes driving very difficult.  
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Have you been to Dr. Stone?  I live way up in northern Vt., and for the first two years I was called crazy.  the vision is the worst.  
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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?
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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!!
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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.
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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.  
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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!
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16462289 tn?1448172323
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.
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The medication RAYOS 1MG HAS HELPED ME TREMENDOUSLY!
An abdominal tumor was removed in Jan. 2015 and it grew back and doubled in size and I was diagnosed with IGG4 inflammatory process in April 2015.   I was on Prednisone 40mg that shrunk the tumor, it was later changed to  methylprednisone due to extreme anxiety and increased heart rate.  I tried Imuran (which caused Hives) and Cellcept (which caused extreme nausea and worse abdominal pain).  These side effects did not begin until 3 weeks after I started these medications.  I went to a new rheumatologist in Dec. 2015 who prescribed Rayos and the abdominal pain is almost gone!  I may have to have 4 infusion treatments of Rituxan .  But the RAYOS has given me so much relief these last few weeks.  Praise & thank God!
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to Wigmar 333.  Please let me know where in Australia you are being treated because my daughter has just been diagnosed with igG4.   We have not yet started any treatment because we are trying to search the best we can do for our daughter in Sydney.  Many medics do not appear to know much about this 'rare' disease and it is very scary and confronting.  Please write back your comments.
Poodle 12 Sydney
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Hi everyone I am Darren from Perth Australia have had my aorta replaced and the top of my femoral artery and lost the main blood supply to my left kidney. The doctors tell me that igg4 did the damage and now it is attacking the ends of my grafts that make up my aorta. My immunologist wants to give me infusions to supress my immune system. if anyone has any information about this it would be greatly appreciated    
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Wow I am so happy to have found this post. I am a 39 year old female from Canada, and I am currently being investigated for iGg4 disease, after my blood work showed an elevated iGg4 level. I am still requiring further bloodwork and scans that are yet to be scheduled. I have suffered for most of my adult life with severe sinusitis/rhinitis, headaches and bilateral growths in my middle ear that prevent me from hearing properly or even travelling by airplane (my ears cannot equalize pressure). I have had tubes in both my ear drums for almost 13 years, but now the growth in the middle ears will not allow for a tube insertion any longer. I have had two major surgeries in both ears to remove the growth/scar tissue, only to have it all grow back in less than two years. My next option is steroids and/or Rituximab, neither of which I am interested to go ahead with at this time. I am now looking at trying a gluten-free and maybe even a full-on anti-inflammatory diet to see if anything will help. Would love to hear form others who have head/neck symptoms like myself, and how things are going for you.
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I am a 24 years old and I was diagnosed IgG4 related disease and celiac a few month ago. I am too very happy when I found other people with this disease! I have IgG4 disease in the nose and sinus. I have been two surgical because of chronic sinusitis. This disease makes the scar on my nose and has damaged my nasal bone. Really I am worried about my future with this terrible disease.

I have gluten-free diet and I use Prednisolone, IgG4-level has lowered little bit during the follow-up. I was surprised many of you commented about celiac or gluten-free diet has helped. Now I have been thinking what would be connection between celiac and igg4 disease... Someone said in this post that her sister's IgG4-level has fallen after gluten-free diet.

If you have IgG4 disease in nose or sinus it would be nice to know what is your situation in this moment.
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How do we reach this Dr Stone I keep hearing about? My sisters boy friend is fighing with this lgg4 and we live in Baltimore and he has been in and out of the hospitals and is on so many pain killers its crazy. Trouble breathing now and the doctors here keep cuting on him its crazy. I can be reached on fb roselubinskiwilkes. Thank u
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How do we reach this Dr Stone I keep hearing about? My sisters boy friend is fighing with this lgg4 and we live in Baltimore and he has been in and out of the hospitals and is on so many pain killers its crazy. Trouble breathing now and the doctors here keep cuting on him its crazy. I can be reached on fb roselubinskiwilkes. Thank u
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IgG4 disease was first recognised in 2001 in Japan. In 2011 it was first recognised as a distinct disease entity.

No one presently has a clue what causes it.

There is a moderately specific histology found on biopsy. The IgG4 levels are elevated in 60-70% of patients. It appears to affect multiple organs. It responds of steroids and not much else. Other drugs have been tried but the effect is not great.

Diagnosis is difficult. It may affect the lymph nodes - especially those in the head and neck. It may affect the sinuses or the lungs. It may affect the oesophagus, the liver, the pancreas, the gall bladder and bile ducts, the skin, the eyes and the joints. It also affects the prostate and the bladder.  It may produce a rare condition known as retroperitoneal fibrosis. It has even been reported affecting the pituitary gland in the brain.

It is highly probable that a number of conditions currently considered to be different are all part of this syndrome.

It is not known to be due to any known virus, bacterium or other infectious agent.

It seems likely that this condition is much more common than we think and that if we look harder for it we will find it.

At the moment it is considered a rare disease and no one has much experience with it.
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I was diagnosed in February 2015 at Cleveland Clinic with IGg4. I have a pseudo tumor located in my thoracic spine from T-2 through T-8.  I was diagnosed by Dr. Elizabeth Ray with the help of Dr Stone from Massachusetts General Hospital. I am one of two known cases in the spine at this time. I have had this since 2005 and numerous surgeries to the spine. I also had to have my right eye removed because of a pseudo tumor that had grown behind my eye due to this disease.  I had a lung biopsy to rule out sarcoidosis.  All of my pathology reports would always come back as chronic inflammation. I have had one infusion of Rituximab and currently take methotrexate after an unsuccessful attempt with Cell Cept. I'm now going back tomorrow to Cleveland Clinic for another MRI. I am having severe back pain again and sweat when I eat from the neck up to the point I am wringing wet. Is anyone out there having these same kind of conditions?
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I have always thought through positive ANA and joint pain and fatigue and extreme dry mouth tinnitus neck pain arthritis psychosis from prednisone depression occipital and trigeminal nerve pain but negative SSA and SSB for sjogren's ...so I went to shands and did tons of work ups and they did a lip biopsy and it came back chronic sclerosis sialadenitis yet I have not one single symptom of it. I am being tested with a blood panel for igg4 and if pos we will contact dr. Stone. I have no life and can't lift anythinf, exercise Etc due to extreme neck arthritis. Basically my head is drying out. My salivary glands are disintegrated. They had to cut under my nerves to find new ones and I now have no feeling in my bottom lip and Constantly get mouth sores in that area. I hAve depression memory problems and psychosis. I was on benlysta infusions for 2 years but want to be on rituxan. I was just on enbrel for joint pain which it helped but dried my head out even more and my tinnitus was louder than my hearing aids and tv. I was a very successful photographer and designer. I am 46 and feel my life is over. No one will help me.
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1530171 tn?1448133193
Hi Rahal.

So sorry about all your suffering.
You might find some pertinent information by opening the previous thread in this community, titled  "Autoimmune Disease".

BTW this is a very old and long post and your reply is at the bottom with little visibility.
Christie has not been on this forum for over 5 years and has only posted twice, so I don't know if your post directed to her, will have much response.


It might be best, if you post this as a new question in various Communities here in MedHelp, so it will be more visible.
Try to title it in a way that it can attract attention like :
" IGg4 DX- Loss of one eye due to pseudo tumor and now...
or " IGg4 -related disease- I'm one of two known cases with pseudo tumor in the spine".  

Excessive sweating when eating may have to do with overacitve T2/T3
nerves responsible for facial hyperhidrosis or inflamed parotid glands.

Look into the numerous benefits of Nigella Sativa Oil and Seeds, which may have multi-organ protective qualities, also anti-tumor action and a lot more!

Best wishes,
Niko


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