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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 go to georgetown pancreatic disease program.  They are very knowledgeable.  I’m in remission based on clean paleo diet.  On no drugs now.
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I have just had my submandibular gland removed to confirm IgG4. I have had swollen lacrimal gland and have numerous eye problems. I have chronic rhinitis multiple lung nodules and many other problems. I cannot find the forum for this disease so replied here. I would be most grateful if anyone can tell me where to go for the general IgG4 forum.
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1530171 tn?1448129593
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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Hi. You can add me to the list of pseudo tumors in gone spine.  I have 6. Been a year now.  How aggressive did the dr's get
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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.
Avatar universal
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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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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I'm sorry to hear about the pain your sisters boyfriend is having.  My husband was diagnosed with IgG4-RSD in 2015 and was made a type 1 diabetic by a surgery he didn't need from this often misdiagnosed disease which surely contributes to the fight his body takes on daily.   He continues to relapse about every 6 months and has only been treated with steroids for the flare ups.   We are trying to get him on Rituximab to see if that treatment will work better because long term use of steroids is problematic in itself.  One bit of advice for those using steroids, START MUCH LOWER!!!  There is no strong study/studies out there of how much to start a patient for disease control (regardless of the disease) and we learned it is effective much lower than many doctors are prescribing.  Question your doctors if your dose is so high.
Regarding medical help in the Baltimore/DC area, I posted a response earlier about Dr. Sean Whelton @ Georgetown University Hospital.  He has the experience with IgG4, if he will see new patients.  Good Luck!  
Avatar universal
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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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 was in the hospital and they found I had IgG4 Feb 2016.  I was given prednisone and rituxin infusions alone with cellcept and viread.  The tumors on my organs did not get any larger and the ones behind and in my eyes shrunk.  I was taken off the prednison and the rituxin was changes to every 6 months.  Now the tumors have gotten a lot bigger and I have non stop presure headaches.  My vision has gotten real bad and the double vision is back.  The doctor has put me back on infusions every 4 months with one two weeks after each of them.  I will be starting the prednison again in a couple weeks after more MRI test are done.  Then the doctors will decide what steps to take next.  I started feeling really good until the prednisone was stopped and rituxin infusions were changed.  
I was in the hospital and they found I had IgG4 Feb 2016.  I was given prednisone and rituxin infusions alone with cellcept and viread.  The tumors on my organs did not get any larger and the ones behind and in my eyes shrunk.  I was taken off the prednison and the rituxin was changes to every 6 months.  Now the tumors have gotten a lot bigger and I have non stop presure headaches.  My vision has gotten real bad and the double vision is back.  The doctor has put me back on infusions every 4 months with one two weeks after each of them.  I will be starting the prednison again in a couple weeks after more MRI test are done.  Then the doctors will decide what steps to take next.  I started feeling really good until the prednisone was stopped and rituxin infusions were changed.  

are you feeling any better? Does the gluten free diet works for you? My mom is in ontario and diagnosis as igg4 after remove the orbital glands from one eye. Her eyes are swellon again, and now extended to other area like salivery gland, sinus, and she has asthma , double vision, lost sense of smell sometimes. She did not try any medication. the dr believed the side effect would make he worse then what it can cured her. However, I am not able to find a specialist in IGG4. Hoped you will feel better!
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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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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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Avatar universal
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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16462289 tn?1448168723
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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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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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 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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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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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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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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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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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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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 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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Avatar universal
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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