I have had a series of inflammatory conditions including but not limited to.. Hashimoto's thyroiditis, thyroid cancer had a partial thyroidectomy, lupus, CNS vasculitis, Sjogren's, AMPPE, fibromyalgia, Crohn's disease, positive P ANCA....I have extremely swollen submandibular glands that none of my doctors seem to be concerned about. They are huge, I look like a chipmunk with walnuts stored in my neck, under my chin...lol...the doctors gave me a month round of prednisone, and 3 months antibiotics with no effect whatsoever. Has anybody had this problem and does anybody have a solution to make it go away???? I know it can be a side effect of autoimmune diseases, especially Sjogren's, but how can I make them go back to normal???.. Please and Thank You!
Extremely swollen submandibular... - Vasculitis UK
Extremely swollen submandibular...
Hi Diane Marie, this is (forgive me) very interesting.
As a former dentist I have come across acute submandibular sialadenitis but there was always an obvious cause (eg a stone in the duct) and it is usually unilateral and not chronic. It's always a mistake to try to make the symptoms fit the disease, but there is an auto-immune disease which has only very recently been recognised. It covers several of your symptoms including Hashimotos, CNS involvement, eye involvement, bile duct problems and pancreatitis (?the Crohns diagnosis). I don't like getting into too much personal detail on open pages so can you message me, telling me how old you are & where you are treated. Or even better, send me an email john.mills@vasculitis.org.uk
hi John thanks for the reply!.. Yes I forgot to mention I also have occasional episodes of pancreatitis, that is why I mention my conditions with adding the 'not limited to' cuz I knew I'd be forgetting somethings!
I am NOT very proficient on emailing, and have never initialized an email, only reply, so please allow me to give y Hospitalou my email address, and I can reply to your message.. diana 55106 at yahoo.com
just quickly adding here, I will be 53 in May, was diagnosed initially with the serious stuff when I was about 35, thyroid cancer was 2006, carotid artery sheath tumor Halo radiation 2007, Sjogren's diagnosis 2015....I also have had about 10 tumors removed in my life, and did have genetic testing for NF1 and NF 2, both negative.
I have been most recently treated at United specialty clinics, at United Hospital at the vasculitis / lupus / arthritis specialties, Saint Paul Minnesota. I have also been treated at the Mayo Clinic, in conjunction with Health Partners clinics, when the doctors were confused why somebody so young as I had all these serious problems including the rare AMPPE, they sent me to the Mayo Clinic to confirm their suspected diagnosis. initially they thought the eye disease was supergenus choroidopathy....spelling?.. But they decided the AMPPEwas correct...
well that was a lot hopefully that sheds a little more light on my situation....
thank you so much for your interest and I eagerly look forward to hear about this condition you mentioned!
Oops...should have spell checked better.. Don't know how the word 'hospital' got between the word 'you' when giving my email...lol
Forget about the email, this will do. I see you are in the US - I am in the UK.
Wow, you have had a bad 10 years!
The disease that I think you should mention to the doctors is called IgG4 disease or IgG4 syndrome. It was only recognised about 10 years ago and is very rare, so not many people know about it. However I do know of patients who have been treated for it at the Mayo Clinic, so that's the place to go.
Pancreatitis is one of the most common manifestations of the disease, but the submandibular swelling is a common feature. The symptoms can mimic Sjogrens and many other diseases. I have a list here of 16 different organs that are frequently involved. It is a disease that causes fibrosis - which results in pseudo-tumors.
It is quite difficult to diagnose so you do need medics who understand the disease and are familiar with it - eg at the Mayo.
The good news is that (if it really is IgG4) it can usually be effectively controlled with steroids and rituxan.
Don't let me raise your hopes too high. I might be totally wrong. A few months ago I had never heard of this disease and I have never come across a real live person with it. That's why your case is very exciting for me (but not for you!).
By coincidence, I am just in the process of getting a doctor with lots of experience of IgG4 to write a page on it for the Vasculitis UK website. The guy who I know who knows most about it Dr Aman Sharma, a very well known rheumatologist in India. I have his new book on vasculitis which has a chapter on IgG4 - otherwise I would know nothing about it.
Best wishes - John & Susan
Well, my goodness thank you so much for that information, very intriguing! I am going to see if I could learn anything about it on my own, to decide what to do.... thank you for mentioning the doctor's name and the condition name, that will help me research. Of course it's all very rare, seems to be what I'm told about all of my conditions! when I was initially diagnosed in my thirties, I was a medical mystery and they told me only three other people in the whole country had everything I had! They wanted me to start high dose of steroids and cytoxan, but I wanted time to think about it... I was still working at the time, and they sent me a fax informing me that one of the three people just died because she too didn't start treatment right away. So basically they scared me into starting treatment...I did it for only nine months, couldn't do it anymore. I had them taper me off and that was the end of treatment for me. They wanted me to start a monthly methotrexate drip, but I decided against it.
Well dear sir, thank you for sharing your knowledge, I should say your EXTENSIVE knowledge, and I sincerely look forward to hearing more about it!
...and as far as you know there's nothing I can do outside of a doctors office to make the glands go back to normal?.......arrggghhh...
I am hoping there is a way for me to read this paper you speak of when it comes to print!
Thanks very much for your time, and sharing your recently learned info!....
I always say... knowledge is power, and sharing your knowledge empowers others!
As I said, I might have got it all wrong - transatlantic diagnosis is not ideal!
If you like, I can photocopy the 10 page chapter on IgG4 from Aman Sharma's textbook. That's probably breach of copyright, but it would only be for your use. I would need a postal address to send to. It would come with the caveat that it is a medical textbook, so although it is very readable compared to most medical textbooks, if you don't know all the medical terminology it might not easy to understand and of course there is the risk that you might read things that you really would prefer not to know!!
There are certainly some decent rereferences on the internet - but stick to reliable ones like NIH, Mayo Clinic or Johns Hopkins.
well John this has proved to be most interesting! I have been researching this condition for over the past hour, and I swear I could be the poster child for the condition! I see it has been treated at the Mayo Clinic, and I am very disappointed when I presented to them almost 2 years ago with GIANT submandibular glands they weren't concerned and didn't have anything to say! They have my entire history so they know about all my other conditions! I even showed them pictures I had taken when they were so large it looked like I had two larger than normal eggs under my skin! Didn't even phase them they didn't seem to care. wondering if the vasculitis clinic I go to knows anything about it?....well you've given me a lot to think about, especially since I've said I'll never do high dose steroids again.. Seems the treatment is steroids and rituximab... I'm thinking for sure I'm going to call the Mayo Clinic on Monday and ask them some questions about my visit... Thank you again John!
Hope your weekend is blessed!
John,
Can you please let me know the full name of the book by Dr Aman Sharma. Is it Textbook of Systemic Vasculitis or he has written a book thereafter. Also Can you please suggest some books that gives insight into this disease from a patient viewpoint ? I've Kidney specific ( till now) vasculitis .
Many Thanks
Mukesh
Hi Mukesh, the book is called "A Textbook of Systemic Vasculitis" . It is edited by Aman Sharma and published by Jaypee Brothers Medical Publishers of New Delhi. It is an excellent book if you are interested in all types of vasculitis.
Do you know the name of the type of vasculitis you have? There are several types that can affect the kidneys. Where do you live?
Best wishes - John
Many Thanks for your prompt reply John. Shall contact you at ur e-mail account for some more clarification.
Just a thought could this be caused by a blockage/obstruction of the salivary ducts? I had this a s a child and had, as I recall, quite a large swelling-in my mouth/neck area.
Hi, thanks for sharing your thoughts, but no, that is definitely not the problem. I've seen five specialists, and had two series of scans done, all with no answers...!
Hi Andrew, you are quite right, but it is usually only one duct that is affected & there are 2 submandibular glands & taking all the other factors into account it might be something bigger.
Wow! Your medical history sounds just like mine! I too get the swollen glands. They hurt like crazy too when I salivate or start to eat. I was told it was the Vasculitis and or Sjogren's. Nothing but time made it go away really. I too was given more steroids and antibiotics. That didn't help.
Will be interesting to see what the new diagnosis is John. I also have had tumors, thyroid removed because of tumors, lupus, RA, CNS Vasculitis, CIDP, Sjogren's, MS and probable Crohn's. I'm getting IVIg which has done wonders for all of it! I got out of my wheelchair/bed and can now walk! I got the IVIg for the CIDP but interestingly enough it helps it all tremendously! Even getting off steroids. Down to 10mg. Hydrocortisone. Yay!
Hope you can find and answer! I would love to know for when mine flare up again. It's happened several times.
Wow, very interesting chatting on this site indeed! Years ago they told me only three people had the same conditions as I had in the whole country! Always wondered what did I ever do to get so much piled on? Wondering if I should go back to the Mayo Clinic or not with this new information from John, which is very interesting, but if I don't want to go on steroids, and that's part of the treatment, why should I bother them?.. I will do some more investigating, see if I can learn anything more about this condition myself. Sorry to hear that you also suffer from similar conditions as I have had to... It certainly has been a long long road of medical history in the making! ....what is the IVIG you mention?I know at one point they wanted me to do a methotrexate drip,.. Wondering if that is what you mean IV..
Very happy for you that your treatment is giving you relief and freedom you deserve!
Blessings and best wishes for you!
Hi Tinabelle,
Where are you treated? Are you in the US or UK?
How old are you and how long have problems been going on.
As I said earlier, I don't like discussing too much of people's personal medical info on the open page so if you want to contact me by message, that's better.
The disease I'm talking about was only recognised about 10 years ago and I've only just learned about it and never come across a real live case until (maybe) dianemarie - so if I've come across 2 cases I will be REALLY excited. (Maybe I should get out more!) It is very rare, even by vasculitis standards! It does cause pseudotumours.
I cannot find any reference to the use of IVIg for IgG4 but looking at the pathology of IgG4, IVIg might have an effect - but the disease is so newl that there's not much treatment evidence yet. It was first recognised in Japan as a disease involving pancreatitis with other problems associated, but more cases are now being identified around the world.
I have been told the same thing. That I have a one in ten million chances of getting this disease combination! I was told I shouldn't even be alive but here I am! LOL. Not going to listen to that nonsense. I had been on high dose starroids, Methotrexate, Cellcept, Orencia and two other biologicals I can't think of the names of right now. I even had eight months of Cytoxan chemotherapy. Almost died three times. My kidneys almost shut down completely but I prayed and made it through. After none of that helped I got the IVIg. This is an infusion of the Ig antibodies from thousands of other people. It is used for treating CIDP, Lupus, MS and some other autoimmune diseases. It has been the best for me.
Blessings to you on your journey to find a good treatment! I too can't wait to hear about what John was saying about a new diagnosis.
Be well and take care!
oMG this is crazy! I have been researching this condition that John mentioned, & I swear I could be the poster child for it! I'm a little bummed out because it seems that preferred treatment is 40 milligrams or more daily of prednisone....from what I've read it seems that you can be tested for the antibodies? hoping its as simple as getting a blood test.. But it seems biopsies might be needed as well.. Argh, I hate those!...wow you and I really do have a lot in common!... I did 9 months of cytoxan whats 100 milligrams of prednisone daily. I gained over 50 pounds from the steroids, and I swear it was all in my face!! That was a very difficult time in my life....well I'm going to do a little more studying on this condition, take some notes, and decide if I want to pursue treatment...
Have a blessed day!
mine has been blessed extensively by this revelation!!
oops sorry I need to spell check better.. Lol, I meant to say 'with' prednisone, not 'whats' prednisone......
Hi John,
I don't mind discussing my diagnoses online either. I think it could maybe help someone someday so I don't mind.
I am being treated in Texas in the United States at an amazing Rheumatologist who has found everything. I had been to the Mayo Clinic when I kept being told it's a mystery but even the Mayo could only say it's some type of autoimmune disease. I've been sick since I was 26 but only properly diagnosed in 2010! I was 45 then. I am now 49.
I am completely intrigued as well! I also have had pancreatitis and currently being treated for pseudo tumor as well! This is completely strange! Even my rheumatologist has been scouring his sources because he also thinks it could be something super rare or even new. He used to be a research physician and is brilliant! it's just the odd combination of so many autoimmune diseases at once. Most people have more than one autoimmune disease but rarely over four. I'm currently sitting at 9 plus other diseases as well.
I completely hate the steroids too! I gained 73 pounds in seven months while I was in the hospital. I had 1000mg. For two weeks then 60mg a day for two years. I also got osteoporosis and broke 4 vertebrae which was the MOST painful thing I have EVER been through. I am now almost completely off steroids. I won't take them again. The side effects are too dangerous for me. I am so thankful for prayer and that my doctor started the IVIg because it has given me my life back!
Please let me know where I can get that book. I have heard of him and would absolutely love to read his book! I have a biomedical degree so I should be okay with the medical terminology.
Thank you!!!
Tina
Hi Tina, you can buy the book from Alibris, which is a US on line second hand bookseller based in California, but I bought my second hand copy for the equivalent of about $50 from the UK Alibris company. It's rather more new, but usually if you buy medical textbooks the price is more like $00s.
If you are interested in all types of vasculitis, as I am, it's the best book I have come across & I use it daily.
I have to put an update on my post! I phoned my doctor's office who had recently done a long string of blood work, & I wondered if they had tested for this IgG4 that John mentioned to me. The nurse said she would talk to the doctor and she will get back to me..she called back a few hours later and said that the doctor sent in the request for the lab work to be done at my convenience! They aren't even making me drive all the way downtown to their facility, they said I can go to my closest Allina clinic, which for me is less than a mile away..
For some reason I am really shocked by this, don't know why I was expecting a roadblock through them! I was half expecting I would need to start out with new doctors. I simply was making the inquiry if the test had been done, so I find it interesting that the doctor went ahead and put through the orders for it without even wanting to see me...
Perhaps she is thinking there might be something to this as well...!
I've done a bunch of reading on it, and it seems that there should be another test called plasma blast count? Anybody know anything about that?..I read that "plasma blast count is a potentially useful biomarker for diagnosis, assessing response to treatment, and determining the time for retreatment".. Thinking I should call back and see if that was included in the new blood workup request...
Oh, I forgot to mention when the nurse asked me initially where I heard about IgG4, I said Aman Sharma, Textbook of Systemic Vasculitis, referred by John at vasculitis UK....maybe it was the name dropping that got the desired results??!! Lol
That's great dianamarie! I'm going to get tested next month with my regular blood work because I too have most all of the symptoms. So strange but it might explain a few things!
Thank you to John for letting us know about this relatively new disease!!
Healing hugs to all!
Tina
Glad you're able to get in for your blood work soon!
I called the hospital clinic today to make sure they had the orders for the blood draw, and she said yes I just need to make an appointment. So I made an appointment for 6:30 this evening, and when I got back to the lab the lady says, "sorry diana, but I am unable to do this request" !!!!! I said please explain! And then she went on to explain that the orders didn't have enough information it simply said "miscellaneous blood draw" IgG4,....and went on to explain that every blood draw is handled differently, what they do with it and where it goes, which of course makes sense. She said I have to call back in the morning to speak with the lab director who will have to research it and try to figure it out, and that she may or may not have to call my doctor for clarification.
Boy oh boy, sometimes I just feel like nothing ever goes right! So now I have to wait on them and drive out there again.... But I always try to find some positive in every negative situation, and for this situation I will be able to inquire about the 'plasma blast count' ....which by the way I mentioned to the lady at the hospital today and she seemed to know what I was talking about because she said that would be a good idea, and I definitely should mention it to the director because she can get stuff done.
So, once again... Until tomorrow... Lol
Also, I have to mention that it seems that my submandibulars, which really were huge, have greatly decreased in size over the past 3 days! They still are larger than normal but not as out of control as they were. I am extremely happy that they seem to be improving, finally,, but this makes me wonder if the condition has to be active for it to show up in the blood work? Or will it be positive regardless of any symptoms or current activity if I indeed have this type of vasculitis. I did a round of prednisone a couple of months ago, so not sure how all this comes into play. Guess I'll need to talk to the doctor after the blood work up to understand it all.
Yes Tina you are correct... many thanks again to John!!...I so agree with you that it would be nice to have something to explain all these conditions and wrap it up under one title!
Blessings & hope
Well I just got a phone call from the hospital lab and they said they called my doctor for clarification on what needs to be done in regards to an IgG4 blood test and to inquire about adding the plasma blast count, as I had asked if it could be added in, and the doctors assistant said they have no idea what a plasma blast count is, and perhaps I should go to the Mayo Clinic to have the tests done regarding it since the blood workup is sent to the Mayo Clinic for the results anyway. How can my specialty clinic say they 'specialize in vasculitis', and yet have no idea about these things? ??....another interesting thing is the lab director said that IgG4 is to detect allergy sensitivities....WHAT????
.....starting to think maybe I do need to go back to the Mayo Clinic for a work up regarding this!
has anybody heard of a 'plasma blast count'?
Nasal detritus problems.
Chicken Pox
Biphosonates ( Alendronic acid etc ) 
Lost sense of smell and taste
