Help with rare diagnosis of IgG4 Sclerosing Disease??

[Christie1983]

I have recently been diagnosed with a rare autoimmune disorder called IgG4 Sclerosing Disease. I know very little about it and have had multiple surgeries and have seen MANY doctors regarding it. So far none of my doctors have seen anything like it and the only reason I reached this diagnosis was because one of my doctors reached out to a colleague at the MayoClinic in Rochester, MN who has 8 patients with this disease. I just want help understanding it and maybe to reach out and speak to anyone else that has this as well.

[wigmar333]

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.

[nutrinut_bob]

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.

[Morgan73]

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.

[nutrinut_bob]

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.

[nutrinut_bob]

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.

[Morgan73]

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.