Promised Update - Possible lymphoma and CNS involvement

[MAT51]

Yesterday I saw the rheumatologist - who sadly is about to retire. He said that the very positive lip biopsy and my immunology and symptoms mean that I have Sjogren's rather than RA as my primary disease. No surprises with this outcome but a certain amount of relief at at last having it confirmed by a specialist of his experience and calibre. He admitted that he rarely gets positive lip biopsy results like mine.

Treatment options seem limited for now because he feels the priority should be to rule out organ involvement before decisions are made about treatment. He is concerned that my worsening disequilibrium might indicate CNS involvement, so has arranged a brain MRI for me. He checked my lymph glands on neck, under arms and groin and found a hard area of swelling on my groin. He is rather concerned that my night sweats and this swelling might signify Lymphoma so I'm to have a full body MRI as well. Because the NHS is very beleaguered now there are long waits for everything including MRIs. So I will have to wait three months for the results from another rheumatologist, as mine will no longer be working there by then.

He said he didn't want to scare me more than necessary but thought it important that I realise that if I do have organ involvement of any sort then I will need a big gun biologic such as Rituximab. He said there is no modifying treatment that would help the small fibre neuropathy of Sjogren's - only symptomatic ones. He said SFN of Sjigrens normally goes so far but then settles at this level and doesn't either improve or progress. This was what the neurologist said too.

So I am concerned that, if nothing shows up on the scans I'll be left unmedicated - having already tried and failed to tolerate four DMARDs and I don't want more steroids. Which takes me to the very perverse position of almost wanting something to show up on the scans so that I can try Rituximab.

I'm so fed up with feeling stoned all the time and having fugue like moments when trying to navigate busy precincts or cross roads or feel I'm falling if I look up or to the side. It used to be a barely perceptible thing for me - just feeling of being slightly out of kilter. But now it's affecting my confidence a fair bit. I understand that he was concerned about this problem signifying CNS involvement but if it doesn't show up in scans then I'm left high and dry with only topical treatments now. But I've never seen an ENT specialist and I have periodic tinnitus now - surely it could be an autoimmune ear problem? Neuro said no because it only affects me when I'm walking or standing.

IViG wasn't mentioned by him and I was so addled by what he was saying about possible lymphoma. I'm not so worried about this myself because there could be lots of other reasons for a swelling on my groin and my neck and armpits were fine.

If anyone has experience of Lynphoma in the groin then I'd be interested to know how it was treated and what symptoms you had. Same with disequilibrium showing up in brain MRI.

[anita]

There are many here that have had lymphoma, so hopefully one of the (or more) will pipe in.  but until you have positive results for this, not worry...as the stress alone that you will be creating can cause you yet more problems.

Being that he is about to retire, he is obviously older...as for why he likely might have omitted IVIG as a possible course of action.  There is a lot of documentation that IVIG can help SFN caused by Sjogren's...especially if caught in earlier stages (but improvement has even been recorded for patients having the disease for longer periods).

There is numerous threads on Neurotalk about IVIG for SFN...even recently discussed at length on this site.

I have CNS involvement including confirmed ganglionopathy...which is likely what he is concerned about.  Sadly, my damage to the dorsal root ganglia is considered permanent...but I have had this for 20+ years that was never treated in early stages (or even in the first 5 years).  The MRI's will be helpful....but I thought you already had MRI's done (at least brain)....am I right?  I don't know if your country has access to the newer MRI/MRN which can look a the dorsal root ganglia or other spinal nerves.  That would be best for you in this situation of possible CNS involvement, but it's still pretty new and rare.

There are many here that have also had Rituxan (Rituximab), so maybe you will get some input from these members as well...or start a thread specifically for this (of course you can search other threads as well).

Does your imaging dept have 'cancellation lists'...where you can be called for last minute cancellations and get in faster.  In the US, we have these lists and it can really speed things up as many people cancel for various reasons and the hospital doesn't want to have open time slots so they call people who have requested to be on this list to see if they are available to come on short notice (usually same day or next day).  it's worth asking about.

Sorry to hear about your news, but try not to fret just yet as you don't have confirmation yet.  Your neuro would have said something had he thought you had ganglionopathy or other CNS involvement.  What did your last MRI show?

Who do you see next?

[warmwaters]

And rituxan is now an option for treating Sjogren's directly, under the new Sjogren's guidelines.  This is very recent info, though people have been using it for a while.

[MAT51]

Thanks Anita. He is at the end of a long career but he was the one who finally worked it all out for me and seemed very on the ball. However I saw a young endocrinologist in the same are of the hospital this morning and he was fabulously up to date so I agree that the incentive to know all about newer developments and research might not be so great.

I'm actually not that worried that I will have Lymphoma. I guess worrying about Multiple Myeloma when the previous rheumy ruled out a connective tissue disease for me in January - but suggested in his letter that I may have malignancy or an endocrine problem - I've given up worrying too much. I'm incredibly tired though because these lousy sweats/ fevers keep waking me and also pomphries on my palm is terribly itchy plus the arthritis in my knuckles is really bad so I can barely fling off the very thin duvet or change my night shirt. Then once I finally manage to cool down I'm cold!

I did have an MRI of my brain 18 months ago in another hospital. He located the results and showed a trainee rheumatologist but I wasn't able to hear what he said properly. It was something about white matter and small vessel disease and my age but as the previous neuro said it was all normal for my 52 years I'm thinking that nothing much will have changed as I had this didequillibrium back then too. He said it was good to repeat these tests every 18 months using a different scanner in this hospital - slight implication that their scanner wasn't so great in the hospital up north!

I really don't like the neurologist here too much though as she just told me to take up tai chi bad get used to the sensation I've lost so far. And she said I looked too well to have a systemic connective tissue disease, which is complimentary if someone knows you well butbthiscperson had never clapped eyes on me before and I felt she should have known better? I see her again mid October and hoping she has changed her attitude thus time. I think the rheumy will fast track the MRIs but he says the next rheum will need the neuro's clinical letter before acting on the information. I guess that they need her to interpret the MRI results and this is why he's putting everything on hold in rheumatology for three months. If I get the MRIs done sooner then maybe I can expedite my next rheumy appointment. They do seem very obliging in this hospital but neurology, rheumatology, gastroenterology and Gynaecology all have massive waiting lists and cancellations are very rare.

[MAT51]

And rituxan is now an option for treating Sjogren's directly, under the new Sjogren's guidelines.  This is very recent info, though people have been using it for a while.

I think it depends which country warmwaters. He did seem to be saying he'd like me to get onto Rituximab but I think they have to make a strong case for funding purposes. I suppose it will depend on who his successor is. I'll fight my corner of I have to but very much hoping, after fighting for so long - that I don't have to.

[Jasper]

Mat51 ..... I am glad you are finally getting some answers.

I know we are in different countries so different rules for drug eligibility apply.

In the US, if a person has RA, either sero-positive or sero-negative, Rituxan is approved for treatment. So, if we have a diagnosis of RA, we can get Rituxan.

As Warmwaters noted, the new Sjogren's treatment guidelines (in the US) do use Rituxan for Sjogren's. However, Rituxan is not FDA approved for treating Sjogren's so Medicare or other government insurance recipients may still have a problem getting it approved for Sjogren's unless they have RA (or some other disease for which it is FDA approved). Private insurance may follow the guidelines and treat people with Sjogren's. Let's hope so.

So, I don't know what the rules are in England (I think that is where you live). If it is approved for RA, then you should be able to get Rituxan under your RA diagnosis.

Rituxan has been shown to help with some cases of neuropathy in Sjogren's, but it is not 100%. Rituxan has helped my neuropathy symptoms but I do not have CNS neuropathy. Mine is sensory polyneuropathy/ganglionopathy and SFN.

I am on Rituxan infusions and they have helped me immensely. I started Rituxan infusions last February and am in the middle of getting my second set of 2 infusions, 2 weeks apart right now. Rituxan has been a miracle for me. It has reduced my fatigue level tremendously and reduced my neuropathy symptoms considerably. 

In the studies, I think IVIg has shown more benefit for neuropathy than Rituxans, especially for CNS involvement.

Rituxan was also shown to be of more benefit to people who have neuropathy related to vasculitis and/or cryoglobinemia. However, Rituxan did help some others with other forms of peripheral neuropathy.

I agree with Anita about the Lymphoma. While it is difficult not to be concerned, there is a good chance you do not have Lymphoma. In addition. my Rheumatologist said (and I have also read this information) that Lymphoma in Sjogren's is very amenable to treatment. In other words, if we have it, it is usually very responsive to treatment.

I hope you can get more answers and some treatment.

[anita]

Thanks Anita. He is at the end of a long career but he was the one who finally worked it all out for me and seemed very on the ball. However I saw a young endocrinologist in the same are of the hospital this morning and he was fabulously up to date so I agree that the incentive to know all about newer developments and research might not be so great.

I'm actually not that worried that I will have Lymphoma. I guess worrying about Multiple Myeloma when the previous rheumy ruled out a connective tissue disease for me in January - but suggested in his letter that I may have malignancy or an endocrine problem - I've given up worrying too much. I'm incredibly tired though because these lousy sweats/ fevers keep waking me and also pomphries on my palm is terribly itchy plus the arthritis in my knuckles is really bad so I can barely fling off the very thin duvet or change my night shirt. Then once I finally manage to cool down I'm cold!

I did have an MRI of my brain 18 months ago in another hospital. He located the results and showed a trainee rheumatologist but I wasn't able to hear what he said properly. It was something about white matter and small vessel disease and my age but as the previous neuro said it was all normal for my 52 years I'm thinking that nothing much will have changed as I had this didequillibrium back then too. He said it was good to repeat these tests every 18 months using a different scanner in this hospital - slight implication that their scanner wasn't so great in the hospital up north!

I really don't like the neurologist here too much though as she just told me to take up tai chi bad get used to the sensation I've lost so far. And she said I looked too well to have a systemic connective tissue disease, which is complimentary if someone knows you well butbthiscperson had never clapped eyes on me before and I felt she should have known better? I see her again mid October and hoping she has changed her attitude thus time. I think the rheumy will fast track the MRIs but he says the next rheum will need the neuro's clinical letter before acting on the information. I guess that they need her to interpret the MRI results and this is why he's putting everything on hold in rheumatology for three months. If I get the MRIs done sooner then maybe I can expedite my next rheumy appointment. They do seem very obliging in this hospital but neurology, rheumatology, gastroenterology and Gynaecology all have massive waiting lists and cancellations are very rare.

Nothing upsets me more than a doctor making some stupid comment about how good I look or that I look too good to be that sick...as if it minimalizes my medical conditions!!  That is so wrong.  I certainly hope she changes her tune next time she sees you and realizes she was incorrect about the seriousness of your condition.  Is there any other neuro at your hospital?

IVIG would be your best bet...more documentation for improvement than Rituxan in regards to SFN/CNS resulting from Sjogren's.  I hope the new one you see after the MRI's are completed will consider this.  Actually, it should be the neuro that orders is...since it would be treating the neurological component of the rheumatic disease...but it could certainly be turned around to treat the root (Sjogren's).  Probably best this way, as your neuro doesn't sound lie she'd be very helpful in ordering IVIG.  Keep in mind that the longer this goes on, the more permanent the damage in regards to CNS/ganglionopathy.  You have already dealt with this for a long time. 

Ah yes, I remember now you mentioning the small vessel disease (age appropriate) on last MRI.  It will be interesting to see if there is any change...likely not, but 18 months is a long time in terms of autoimmune process.  it certainly needs to be repeated at this stage and with your new Dx.  I hope they can get you in soon.

Can you describe your neuro symptoms...and are they treating you symptomatically at this point at all?

[MAT51]

Anita my symptoms are mainly a type of dizziness that the neuro said is disequillibrium. For ages it has been quite subtle and similar to something I suffered in my late 20s for a while - just not feeling secure in my footing and struggling with doorways and navigation if there's no natural lighting. I suppose the feeling is of being mildly stoned all the time - without the pleasure - or in my case nausea and vomitting, as pot makes me very sick! It only affects me when I'm standing up or walking though - not a benign proximal vertigo type of thing at all. The neuro said this doesn't tarry with vestibular problems because it only occurs when I'm on my feet. But increasingly I find I have mild tinnitus and sound sensitivity and aslight motion sensation when seated too - but almost imperceptible so nothing like the walking trouble at all.

The only time that I really have the vertigo is if I look up at something - and then I feel I'm falling into space. And I generally do then lose my balance. Otherwise it's more subtle still but constant and I feel I have to constantly be on my guard when things around me are moving ie people, bikes, vehicles. If I have to turn round to look at someone or something then I need at least ten seconds to recompose myself - or rather realign. Like when a computer or tv slowly starts going out of focus I feel this symptom is slowly but surely getting worse - although it was severe when I was in New York a few years ago but that was because of the jet lag I think.

I actually had one of my moments the other day when the rheum pulled the curtain round me so I could pull my pants back up and get my shoes on. He carried on speaking and expected me to come back through the curtains but I panicked and couldn't get out for a moment and stumbled through with the feeling I would topple having bent down to push my dandies on. So he said sorry to me but it was a reality check for him and the junior doctor - as my husband observed. There's usually no natural lighting in these places and strip lights are the worst for triggering my disorientation I suppose.

The neurologist told me it was all about my proprioception and told me I needed to retrain my brain and take more exercise such as yoga and tai chi. She said it wasn't bad enough to warrant neuro physiotherapy. I've done these type of exercise classes in the past but am too busy and unsettled to commit to classes etc presently.  I then failed my Romberg's as I invariably do and she observed some toe/ heel clumsiness - but said in her letter that she wasn't very concerned about either of these things.

So her letter was read by the rheumy and he asked me if I'd explained the extent of my troubles properly. I told him that yes I had but at this stage (April) she was fixed on the idea that my RA was the only problem and as this has remitted it wasn't progressive nerve damage - just leftover from early RA. She told me I was a lucky woman for having such mild RA. I asked her if she knew much about Sjogrens at the start of the consultation but she just waved her hand at me and said "of course I do - I see a lot of this! Not your problem though as you have RA and your bloods and tests don't support the idea of Sjogren's". The rheumy may have picked this attitude up from her letter and my brief account so I'm hoping her attitude will have changed now. I just said to him that she didn't have the latest blood results or lip biopsy so was going by previous rheumy who said I didn't have a connective tissue disease in his February letter. But she's a stubborn young-ish school prefect type so I won't hold my breath that she will change her perspective now!

[MAT51]

Mat51 ..... I am glad you are finally getting some answers.

I know we are in different countries so different rules for drug eligibility apply.

In the US, if a person has RA, either sero-positive or sero-negative, Rituxan is approved for treatment. So, if we have a diagnosis of RA, we can get Rituxan.

As Warmwaters noted, the new Sjogren's treatment guidelines (in the US) do use Rituxan for Sjogren's. However, Rituxan is not FDA approved for treating Sjogren's so Medicare or other government insurance recipients may still have a problem getting it approved for Sjogren's unless they have RA (or some other disease for which it is FDA approved). Private insurance may follow the guidelines and treat people with Sjogren's. Let's hope so.

So, I don't know what the rules are in England (I think that is where you live). If it is approved for RA, then you should be able to get Rituxan under your RA diagnosis.

Rituxan has been shown to help with some cases of neuropathy in Sjogren's, but it is not 100%. Rituxan has helped my neuropathy symptoms but I do not have CNS neuropathy. Mine is sensory polyneuropathy/ganglionopathy and SFN.

I am on Rituxan infusions and they have helped me immensely. I started Rituxan infusions last February and am in the middle of getting my second set of 2 infusions, 2 weeks apart right now. Rituxan has been a miracle for me. It has reduced my fatigue level tremendously and reduced my neuropathy symptoms considerably. 

In the studies, I think IVIg has shown more benefit for neuropathy than Rituxans, especially for CNS involvement.

Rituxan was also shown to be of more benefit to people who have neuropathy related to vasculitis and/or cryoglobinemia. However, Rituxan did help some others with other forms of peripheral neuropathy.

I agree with Anita about the Lymphoma. While it is difficult not to be concerned, there is a good chance you do not have Lymphoma. In addition. my Rheumatologist said (and I have also read this information) that Lymphoma in Sjogren's is very amenable to treatment. In other words, if we have it, it is usually very responsive to treatment.

I hope you can get more answers and some treatment.

Thanks Jasper - all good to know. I'm actually in Scotland rather than England so they have a different set of criteria for funding. But my Sjogrens was diagnosed using the ACR and EULAR criteria so it is all pretty similar. Good to know that Rituxan is working so well for you. Very grateful to you and others for talking me through this even if we are in different parts of the globe.

[Tharrell]

Oh my goodness MAT! Your description of your dizziness stuff! Those were my first presenting symptoms and it drove me nuts not beeing able to accurately describe it to the neuros! I had to leave two neuros in the dust, one was actually the chief neuro of the clinic! ENT doctors also found no problems. My current neuro at the very first visit, after asking me tons of questions, diagnosed me with acephalgic migraines based on my migraine history. We started on norytriptiline which took care of the dizziness/stoned feeling. When that stopped working we switched to topiramate which I still take. She also found that I was B6 deficient and had me start supplementing it plus a double dose of B2. It was such a relieve to no longer have that dizzy/stoned feeling! I still haven't figured out how I can have acephalgic migraine that  literally never stops! Ever without medication! Unfortunately the past 6 month my balance issues, just like you describe, are getting worse! I'm constantly keeling over, but manage not to fall. Thankfully I'm using a cane currently because of my hip. My neuro doesn's take that serious either. I mean how accurate is taking a few steps back and forth in the exam room when the problem is in a normal environment with people and moving things, light changes, normal walking, sudden turns? They always forget about that! Same thing with the eye doctor! I think some of the balance and movement issues I have is due to my eyes. I never see well, which is partially due to dry eyes, and mostly I just see blurry or double. Yet when I do my vision check ups there is not much change. Again, in a normal environment I'm dealing with moving things, different lighting, textures. My brain just can't handle it. So everything is normal. Yeah right!

[MAT51]

Oh my goodness MAT! Your description of your dizziness stuff! Those were my first presenting symptoms and it drove me nuts not beeing able to accurately describe it to the neuros! I had to leave two neuros in the dust, one was actually the chief neuro of the clinic! ENT doctors also found no problems. My current neuro at the very first visit, after asking me tons of questions, diagnosed me with acephalgic migraines based on my migraine history. We started on norytriptiline which took care of the dizziness/stoned feeling. When that stopped working we switched to topiramate which I still take. She also found that I was B6 deficient and had me start supplementing it plus a double dose of B2. It was such a relieve to no longer have that dizzy/stoned feeling! I still haven't figured out how I can have acephalgic migraine that  literally never stops! Ever without medication! Unfortunately the past 6 month my balance issues, just like you describe, are getting worse! I'm constantly keeling over, but manage not to fall. Thankfully I'm using a cane currently because of my hip. My neuro doesn's take that serious either. I mean how accurate is taking a few steps back and forth in the exam room when the problem is in a normal environment with people and moving things, light changes, normal walking, sudden turns? They always forget about that! Same thing with the eye doctor! I think some of the balance and movement issues I have is due to my eyes. I never see well, which is partially due to dry eyes, and mostly I just see blurry or double. Yet when I do my vision check ups there is not much change. Again, in a normal environment I'm dealing with moving things, different lighting, textures. My brain just can't handle it. So everything is normal. Yeah right!

Oh dear Tharrell. Sorry we share these horrid symptoms. Both neurologists dismissed the idea that mine was any type of megraine though because I don't get headaches very often - and never migraine ones. I do wonder if mine is ear related as I have two profoundly deaf sisters and small passages with nose bleeds in winter etc. But neuro dismissed this too as I have a very diffuse SFN which this wouldn't explain.

For Anita - I've just phoned the MRI/ X-ray department and was told that I will get dates soon and this will be done next month before we go back up north on 24th. Phew!

[anita]

MAT,

Your account of the dizziness is clearly lack of proprioception.  I have problems with this as well.  Did he test you for this...by having you close your eyes as he moves your toe up/down...to see if you can tell which way he moved it?  They also check at the ankles and knees to see how far up/severe the problem is.  Of course they can check fingers, wrists, and arms as well.  But the feet are more problematic as it affects your walking.

I HAVE TO look at my feet when I walk, or I will fall.  It is my own way of compensating and retraining my brain to work around the problem.  Have you tried this?  If I lose sight of my feet I have problems...as for why you have problems when you look up.  The same applies in dark areas (because you cannot maintain visual contact with the ground and your surroundings).  There is no real 'treatment' for this other than retraining and compensation.  But it is yet another sign of your neuro manifestations of Sjogren's.

[MAT51]

Yes Anita this is what the latest neurologist told me but she said it wouldn't get worse or get better. She explained that I won't get the sensation back in my feet and I must adapt and learn to live with it. So I've really tried to - although I haven't been able to enrol in any yoga or tai chi classes as she suggested, because we have been moving about and life has been too unsettled.

But the thing is the disequilibrium is getting significantly worse now and I seem to have these fugue-like moments where I just feel paralysed by indecision about how to move forwards or regather my equilibrium enough to cross a road or walk up steps. So if it's caused by propriception problems as you say - then why does it seem to be getting worse?  I'm told my SFN is not progressing and then rheumy said that Sjogren's neuropathy isn't usually progressive after a certain point, but surely this worsening disequilibrium indicates that mine is continuing to flourish? I wish it all made sense to me but it just doesn't!

[irish]

I don't know if you have SFN but it doesn't sound like you have a proprioceptive disorder. I first started having these symptoms back in 1990. I would drive to work in the morning and when I got to work I would have to look at the ground to know where to put my feet. I also would have what I would call ataxia. At this time I had been having issues for almost 30 years and had just been diagnosed with hypothyroid. I did a lot of research on hypothyroid and I did see the words ataxia and proprioceptive disorder mentioned.

I mentioned this to several doctors and they paid no attention. The next thing I noticed that was proprioceptive in nature was really weird to me but did make sense when I thought about it. When I drove home from work at night and drove into the garage which had no automatic ceiling lights, I would reach to open the car door and I couldn't find the door handle. Now, we can't find our car handles the first weeks of adjusting to a new car, but once our brain is trained to our car and the location of the door handle it should be an automatic reflex.

But, it is not, unless you have light. Then the biggest thing I did that was funny I will have to admit, it walking down the hall in the dark to my bedroom. My hubby was sleeping and I could not tell where the heck I was. I felt dizzy and out of it and finally stopped walking and hollered and woke my hubby. "Turn on the lights, I don't know where I am". "What in the heck are you talking about?"

Hubby turned on the light and here I found myself in a bedroom with my nose about 1/8 inch from a door that was all the way open. Hubby couldn't believe that and neither could I. Then there is the walking day or night and always having to watch where the steps, curbs and my feet are or I could break an ankle.

I would pursue this with your doctor and also if you are having a hard time getting treatment you might want to get second opinion. The one thing you can do to help yourself is to keep your legs and arm muscles strong so that you are more sure footed and able to help yourself with both leg and arm strength. It is really a big help. You can get therabands at Walmart for exercise while sitting in a chair. Just some simple exercises a couple of times a day can even help with abdominal strength. Good luck. Irish

[MAT51]

I do indeed have a very widespread SFN Irish - and the disequillibrium started a while after the SFN. When I visited NYC a few years ago it was really bad because of the jet lag. We went up the Rockerfeller at midnight and the blackness of the roof was like a vast black chasm. I gave up with img a minute and just sat down on the ground. A lovely attendant came and chatted to me and gave me his arm and took me round the edges showing me all the landmarks. He seemed to understand where my husband and son had been too wowed by the amazing view that they forgot to think how this vast blackness and lit up skyline might affect me! 

So are your issues not proprioceptive then? I live in Scotland so we don't have Warlmart!