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.
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.
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!
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.
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.
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.