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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.
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,
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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I was diagnosed in March of this year with Igg4. Just finished 7 months of prednisone treatment which took care of all the inflammation in my kidney and liver. Doc tells me to expect an MRI every 3 months to see how things are going. Unsure of what is next for me...feeling great right now off prednisone almost 2 weeks. Hate the drug but love what it did for my illness
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.
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!
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!
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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
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....
I forgot to add that he is pretty much bed bound. I can't even explain just how sick he is....
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?
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>
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>
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.
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.
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
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.
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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