Just diagnosed with IgG4 disease - talk to me!!

Hi

Glad you are feeling better and have an answer. 

How were you diagnosed? Did you have high serum igg4?  

Hi. The lachrimal gland swelling was the final clue. I happened to be on holiday in India and saw an ophthalmologist when I was there. She said it may be IGg4 almost instantly. In summary blood tests (high Igg4)and histopathology from an eye biopsy confirmed the diagnosis. Incidentally just today they gave me results from a re-examined lymph node biopsy taken a decade ago. It also is suggestive of Igg4. Its extremely rare and even a lot of immunologists don’t know much about it. Good luck!

Thank you. Very interesting. I am trying to get a diagnosis but my normal serum igg4 is a problem. I have polyneuropathy and the neurologist is going to do a nerve biopsy to get more clues. I have the sinus, dry eyes and thyroid issues also. They looked for Sjogrens first but negative blood tests. It’s a long hard road eh?!

The problem with getting a diagnosis is that most specialists nowadays are very focused on their own area of expertise. Also interpreting Igg4 results is not straightforward. It’s not black and white as the levels are dependent on the so called burden of disease. If you have a large mass in your abdomen infiltrated with Igg4 you can have very high levels. However if smaller organs are affected it may still be Igg4 related but serum levels can be normal. You really need to see a super specialist to be sure. It’s very complex. I shifted between ENT, immunology and then finally ophthalmology before I was diagnosed.  It took years.  Good luck.  

Thank you Naresh. I share your thinking on the serum level and the blinkered specialists. I have now seen all disciplines and they share a very secular focus! I will use your comments in my Neurology consult this arvo. 

Hi Deirdre 44070, naresh3, and nick72107, I share all of your concerns re team of docs, treatment, etc. I was diagnosed for sure about 2 weeks ago. Back in April I had my 3rd stroke and on those scans the amazing radiologist, God Bless him/her, noted tumors or swelling on both lacrimal glands and with my noted history recommended I be tested for IgG4.  WHAT? WTH is THAT? In hospital it was blown off. They were only focused on the stroke and my heart. "Oh you can follow up with that when you get out", is what I heard.  Guess what? Cuz no one knows what IgG4 is, right!!! So bcuz of my vision from the stroke I go to the eye dr. I mention the lacrimal tumors to him. He says I need to see a "neuro-opthalmalogist", I have never heard of one of those but o.k. From a small town in DE up to Wills Eye Inst in Phili we go! We ask him about the IgG4, like you say, he looks at us like we have 2 heads and asks where we hear about that and we tell him about the stroke scans. He does his own CT of the lacrimal gland then orders the blood test for the IgG4. In the meantime I have a biopsy of the gland. Now I look like Rocky, like I've been punched in the eye and it feels like something is on the back side of my eyelid scratching my eye when I blink. I'm guessing its from the dry eye? And yes, the IgG4 level is way high, the biopsy comes back, no cancer, no lymphoma, its the eosinophil pseudotumor thing with the IgG4 high serum level (I think is what it all is, I haven't seen the biopsy report yet, I see him on the 2nd) So I'm kind of in the same boat, trying to figure out who treats all this, the neuro-op can treat the lacrimal tumors, BTW not only is the IgG4 rare but I also have the lacrimal gland tumors bilaterally, which is also rare! Why the heck not! HAHA But so IgG4 can also be treated by my rheumatologist (I have been dx with fibromyalgia for like 20 yrs, but is it?) Then I'm reading maybe I should go to an immunologist? I don't have one of those yet. But #1 who will follow the tumor to be sure its shrinking and/or doesn't come back. #2 Who is the one to follow the IgG4 in general, to treat but also to follow if it flares or hits another organ, or #3 do you just wait to see what organ it hits next? I already have heart issues, I'm waiting to get a "loop" internal monitor to see if I have A-fib. I've had 1 heart attack, 3 strokes. I have stage 2 kidney disease, now wondering if the IgG4 is to blame. The only good news is the neuro-op thinks maybe the IgG4/Lacrimal tumors may have caused the strokes bcuz they were all in the occipital lobe.  I guess my biggest concern it monitoring?  Sorry this is so long, its so complicated!

Hi my care is now coordinated by my immunologist who communicates with my ophthalmologist and family Doctor. It’s a tricky diagnosis to get even in high tech medicine countries like the U.K. and USA. My condition was actually diagnosed in India and is now being dealt with in the U.K. where I live.  I think an immunologist is probably the most important specialist in a way because this is a multi organ autoimmune disorder. They can instigate treatment and organise monitoring through scans and blood work. 

I have it in lacrimal glands biopsies done need constant eye drops but manage just fine , hope you are well . All the best

Has the lacrimal gland infiltration caused you to have dry eyes requiring constant drops, etc.? My opthamologist says that it is nothing to do with my positive biopsy of IGG4 . I did not have dry eyes before i developed this disease

Hi Eimaikala1, yes I did have dry eyes and my rheumatologist and neuro-opthamologist said it WAS part of it. The lacrimal gland is what produces your tears, how could it NOT be related?! I do have OTC "tear" drops to use but since treatment don't have such a big problem so much, only every now and then. Have you done any research on lacrimal gland and IgG4? I'm a couch researcher for any issue I have, LOL. I want to know everything I can about everything I have 😃

yes like you I do loads of research. and READ a lot about the disease

So i find it unbelievable when the various opthamologists i have seen since diagnosis insist dry eyes s nothing to do with my biopsied lacrimal gland which showed extensive sclerotic infiltration and like my removed and biopsied sub mandibulst gland positive IGG4

So i find it reassuring that your advice is different. There is no research at all in LONDON on igg4 and the eye,

It is so difficult to be a patient when the consultants do not believe dry eye is anything to so with my lacrimal gland infiltration

Wish you good luck. I started high dose steroids about 5 months ago starting at 40mg and very slowly tailing down. I’m on 10mg now to eventually go down to 5mg. I’m sure you will start to feel much better soon. My eyes were very badly swollen and are much better now as is my fatigue. Make sure they do full body CT scans to ensure no other organs involved. 

Thank you, its all a bit of shock but relief at the same time. Seems to be a bit of lonely road with this illness, not finding much out there in the field of support groups etc. Fingers crossed i start to get the benefit soon. Glad you are feeling better and wishing you all the best too.

hi ellie its joe hear could u tell me were consultant was from as iam in Ireland and looking for a consultant and willing to go to the uk to meet the right person thanks

 

Hi Joe, He is called Dr Malaviya, consultant rheumatologist. He practices at Addenbrookes (CUH) & Newmarket Hospital. Also does private consultancy around the cambridgeshire area. he is a very understanding and thorough Dr. Good luck 

Thanks Ellie ,, there some good Facebook pages to talk to people one of them is called ,, living with igg-4 related diease

Hi Joe

I am managed by consultant immunologists in Belfast if that’s any help.  

That naresh ,, do u live in the north as iam original from co, down and now living in Tipperary 

Naresh sorry what's the name of the consultant thanks

Hi I’m under the care of Dr Lisa Devlin and her colleague Dr David Edgar at RVH Belfast

Both immunologists. For my eyes I see Mr Steve White at RVH. Between them they are doing a great job. However to be fair the diagnosis was originally made when I was on holiday in India and by chance saw an ophthalmologist there who thought it was Igg4.  

Yes I live in Co Down. 

Naresh iam from ballymartin,, in codown ,, iam to meet my oncologist on Monday to see what next as he is in cuh in cork and what to do next ,, as the when I spoke to him 2 weeks ago he was going to fine some one so thanks for the names of your doctors ,, If he has not don someone ,, I could tell him about the people in in the royal thanks naresh

Thanks Joe. My docs are good but even they didn’t know too much about the condition and have had to read up to see what to do. It’s also difficult to decide who the specialist should be as it affects so many organs. There are a few  experts in big U.K. centres in London and Cambridge etc. Good luck. 

Thanks naresh

I am under a lupus specialist in a big teaching hospital in London who has an interest in IGG4 and a cluster of diagnosed patients

i have it in orbits and glands, diagnosed by biopsy . i get very tired but have always presumed it is getting older. im in

LONDON

How is your present health? I don't take meds, too frightened of high glucose with steroids, as am diabetic.

i have severe dry eye , do you?