Clarification over diagnostic terms?

[MAT51]

On a different forum I read people describing their Sjogren's as A or B. I queried this and received the suggestion that, as I don't have specific blood markers for SJS, mine might be MCTD instead?

As many of you know my rheumatologist diagnosed me with primary SS a month ago on the strength of my lip biopsy result. So I've been assuming that this makes me seropositive for Sjogren's. I believe my ANA pattern (1:320 nucleolar) points to Scleroderma or Polymyositis - but I don't have the symptoms of these. I was also told a while ago that MCTD usually involves having several connective tissue diseases relatively mildly, rather than one or more severely. And also that MCTD is diagnosed by specific autoantibody results. I know it's not that important to have one disease to blame everything on and the treatments overlap a lot anyway. But, having spent six years feeling a seronegative RA diagnosis didn't fit, I find it does matter to me that I now have the right disease title for me. So is mine seropositive or negative can anyone tell me? And what's with the A and B thing?

Ps Still no news about CT or MRI. Waiting impatiently for a letter to clarify all is okay now.

[anita]

The Sjogren's A & B people are referring to are in regards to the lab test.  Anti-Ro/SSA & Anti-La/SSB are the two labs used to diagnose Sjogren's via the antigen markers.

People don't 'have' Sjogren's A or Sjogren's B...they would either 'test' positive for SSA or SSB...or neither.  up to 40% of patients are sero-negative via blood test.  But that doesn't mean they don't have Sjogren's.  I am sero-negative, yet my lip biopsy is very positive.

Your positive lip biopsy, does NOT make you sero-positive for Sjogren's.  The only thing that makes one sero-positive is to actually have the positive lab makers.  And people don't have to have both markers to be sero-positive...just one.

Have you ever had the labs drawn for SSA & SSB?

As we have discussed before, the strength of the biopsy does not constitute the primary vs secondary Sjogren's.  It only comes down to whether you have another autoimmune disease...primary if the only one and secondary if patient has more than one. Being that you are sero-negative for RA (I always thought you were positive), that may be why they are considering your SjS primary (not counting your RA as an active process).  Primary and Secondary is not treated any different, as both can be mild or severe and are treated based upon the symptoms and organ involvement.

Hope all this helps.  If you were not tested for SSA & SSB before, I'm sure they will now, just for more documentation (but keep in mind that even if negative, your positive biopsy trumps the negative lab).

[MAT51]

Yes it all helps Anita. I'm a firm believer that knowledge is power - or at least gives one a bit more perspective anyway. My immunology panel (ENA) was negative in June apart from the ANA 1:320 with the nucleolar pattern and the IgG and IgA. This means I was negative for everything including Ro & La and RF.

The point about the RA is that it isn't real RA because RA would have come back and be causing erosive damage by now if it was the real deal. I was diagnosed in 2011 with clear synovitis in my MCP and PIP joints on both hands and wrists and some in my metatarsal joints of feet. Personally I think the methotrexate injections for two years plus Plaquenil for 18 months chased it off. In 2011 when diagnosed my Rheumatoid Factor was a weak positive of around 24/25 but the rheumy said this didn't count for anything. He diagnosed me by seeing and feeling swollen synovial joints and my highly fluctuating ESR.

My ANA wasn't tested at that stage but a few years later the ANA was equivocal - DSNA and RF negative. My rheumy called my RA seronegative because my anti CCP was negative. I'm also ANCA negative. The one thing that has always been very high is my sed rate and often my CRP. These still are very high but the new rheumy explained that this is often the case with people with primary  Sjogren's because our blood is much more intense from lack of moisture/ dehydration,  so it is not a good way of monitoring us for active disease as it is for RA and Lupus. It was my high inflammatory markers that convinced my GP and rheumy that my RA was still active for a few years, even though the joint pain had been replaced by very painful SFN.

The new rheumy said that in his view I had only suffered from secondary, non erosive RA for a few years as part of my longstanding, primary Sjogren's. He felt that the RA was just a secondary manifestation of my Sjogren's because of the lack of erosive damage in my joints. My instincts say that he is spot on.

I know so much more about RA than I do about SJS - and it is often said that seronegative RA tends to be less damaging. However I do also know exceptions to this - including one person who was lip biopsy positive for Sjogren's. The bad thing about being seronegative in any CT disease is that it takes longer to diagnose usually but also that the drugs can be less effective. But I just wondered if the prognosis for seronegative Sjogren's is thought to be better than for those in whom it shows up in blood?

I realise that you are an example of this not being the case but I also know that you have PsA so this could be responsible for a lot of your serious disease damage rather than Sjogren's could it not? I have good friends with PsA and also a first cousin so I know what an aggressive disease this is for many.

[nannysbaby]

You may have already seen this information, but it brings out that primay Sjogren's sufferers have an increased disease burden.

http://www.medpagetoday.com/MeetingCoverage/BSR/38713

[anita]

WOW...this is just so weird.  I swear you are a carbon copy of me (medically speaking).  Every time you bring up a condition or symptom, I can relate...because I have already dealt with that particular issue.  It's like you are me a few years later.  Once again, I can relate.

As you know, I have been Dx with inflammatory spondyloarthropathy (Psoriatic arthritis)...directly resulting from the Sjogren's.  I had the Sjogren's first and then recently got the PsA.     

The typical joint pain from Sjogren's was not only getting worse in last 5 years, but I also started getting profound swelling of fingers (sausage digits, as they call them) as well as the knees and hips.  My rheumy/neuro (Birnbaum) had the Hopkins arthritis center do ultrasound on my MCP, PIP, & DIP joints to confirm this new Dx of inflammatory spondyloarthropathy.   They did, in fact, find significant fluid in all these joints...even the DIP (very tips) of several fingers.  Once again, my lab markers were negative, but the fluid proved that I did have this condition.  I also have the very elevated SED rate (100+).  Have you ever had your MCP, PIP, & DIP joints checked with ultrasound??

I'd put money down that you also have inflammatory spondyloarthropathy from Sjogren's...not RA   Your Sjogren's is likely the root of all your problems...so classic is your presentation (as was mine, but no one was wise enough to put it all together until I saw Birnbaum).

The prognosis for sero-negative Sjogren's is the same as sero-positive according to Birnbaum...just as Primary and Secondary Sjogren's can carry the same levels of severity.  Being that my PsA came 15-20 years after my Sjogren's, I'm pretty certain my damage mostly came from the Sjogren's...except for newer joint damage in knees, feet/ankle hips and hands (to include new bone marrow edema in hips).  That I would attribute to the PsA.

[MAT51]

Well I do feel a great sense of kinship with you Anita from across the pond! How I envy your Birnbaum though. I feel that here Sjogren's is seen as a secondary nuisance to the big gun rheumatic diseases and my RA has now been passed over. But then I was treated using dual therapy of Hydroxichloraquine and Methotrexate for a few years so this more or less sorted out the synovitis. Slowly I feel it is returning but not in the way of fractured-feeling in joints or the hot, bruised looking MCP and PIP joints. And of course, the new rheumy has never seen my hands or wrists the way they were then so hasn't the same reference point as my first rheumy - although I've shown photos.

What I think is that I won't be treated properly again unless my RA returns or psoriasis shows up or a Vasculitis rash - some visible clues emerge. This is what a professor of connective tissue diseases told me a few years ago when I sought a second opinion from him. He said its all got to be evidenced in the skin and joints. The reason my systemic dryness wouldn't be treated with big gun drugs, he explained, is because they aren't effective. He said my SFN was Raynaud's and my skin problems were allergic rashes, my high sed rate was autoimmunity and CRP was the test to monitor active disease or infection from immunesuppresants. I was on methotrexate injections and nifedipine when I saw him though. Somehow I find it all very depressing just now but I'm extra fatigued and we are relocating once more so plenty stress! X

[MAT51]

You may have already seen this information, but it brings out that primay Sjogren's sufferers have an increased disease burden.

http://www.medpagetoday.com/MeetingCoverage/BSR/38713

No I hadn't read this before thanks very much for posting it. Q: why does it say that primary SJS is a common rheumatic disease here where other places it's described as rare? Also why is it often presumed relatively benign compared to Lupus or RA. Mine doesn't feel at all benign?! Sorry - not asking you this question specifically nannysbaby but this article made me wonder. I found it very depressing I must say. I hope more research is done soon.

[anita]

I must agree to some extent.  Some doctors are really hung up about joint, skin, and lab confirmation....they want to SEE the disease process.  So those of us who are sero-negative have a harder time getting treatment...even though our disease process is just as severe.  Even the neuropathy is hard to get Dx'd for this same reason (can't see it).

That all being said, I think you have enough going on that your doctors will take this seriously and treat you accordingly.  The vertigo alone is quite disabling.  I also think your skin biopsy would come back positive now, with your increased pain/symptoms of neuropathy.

But that doctor is correct about even the big guns not helping the dryness.  I'm not sure why this is the case, but most treatments are just not that effective at improving the dryness...which is, of course,the core/root of the problem.   However, those treatments are good at organ involvement and neuropathy.

I thought you were DONE moving!!  OMG, your body does not need the extra stress right now. I hope this move is just short distance/local and you can get through this one relatively easily.   I assume you haven't heard about the MRI or CT??

[Tharrell]

Hey MAT, on your mctd question. You do need high anti RNP antibodies and ANA with that as I do. My sjogren's diagnosis comes from the positive ss-B and is part of the mctd. I do have a lot of cross over symptoms, but I wouldn't say that the ra part of it is mild. I was finally given orencia injections after almost two years of begging and complaining about my bilateral joint pain which was successfully treated with Humira before an interim doctor took it away from me.

[MAT51]

Hey MAT, on your mctd question. You do need high anti RNP antibodies and ANA with that as I do. My sjogren's diagnosis comes from the positive ss-B and is part of the mctd. I do have a lot of cross over symptoms, but I wouldn't say that the ra part of it is mild. I was finally given orencia injections after almost two years of begging and complaining about my bilateral joint pain which was successfully treated with Humira before an interim doctor took it away from me.

Hi Tharrell. I only meant that MCTD is thought by some people to comprise of milder versions of several CT diseases. In my experience these generalisations are often the opposite to the reality though.

My RA was never described as mild. I was put straight onto Methotrexate and Plaquenil for them. I had lots of physio and splints and couldn't drive or write or weight bare for about a year. I even got stuck once in the bath because of bad shoulders and wrists, whilst on holiday, when my husband and sons were out shopping. I had to put every ounce of effort into flinging myself out finally, landing like a beached whale on the mat and taking half the contents of the bath with me!

But my Sjogren's doesn't feel at all mild to me either. It's not the eyes, mouth, dry throat/larynx or even the nerve pain that disables me most these days. It's the constipation, pelvic pain, mouth/ nose/ eye pressure, breathlessness, temperature swings and the vertigo/ disequillibrium that I'm struggling with most. Oh and the fatigue - which I never suffered from at all when my RA was in full flood.

I'd love to be able to try Cellcept but we will see whether I qualify or not. I have a tendency to underplay things when I'm with specialists. I recall apologising to my first rheumy for moaning about how my RA symptoms were turning my life upside down. He looked at me and said "it's quite a reasonable thing to moan about I feel - but actually you've hardly complained at all!". My late mum always shouted at me if I complained of pain or unwellness so my instinct is to be stoical at all costs now.

I think the Sjogrens has actually affected me since I was quite young - about 9 years old - when I first started needing eye ointments and all my hair fell out and my teeth were a constant problem. For a while RA even seemed like a welcome diversion from the all over dryness I'd suffered for all of my adult and adolescent life!

This is why i wanted to try and work out what the prognosis might be for the type of Sjogrens that's affecting me. My ANA is positive - but not my ENA.

[MAT51]

I must agree to some extent.  Some doctors are really hung up about joint, skin, and lab confirmation....they want to SEE the disease process.  So those of us who are sero-negative have a harder time getting treatment...even though our disease process is just as severe.  Even the neuropathy is hard to get Dx'd for this same reason (can't see it).

That all being said, I think you have enough going on that your doctors will take this seriously and treat you accordingly.  The vertigo alone is quite disabling.  I also think your skin biopsy would come back positive now, with your increased pain/symptoms of neuropathy.

But that doctor is correct about even the big guns not helping the dryness.  I'm not sure why this is the case, but most treatments are just not that effective at improving the dryness...which is, of course,the core/root of the problem.   However, those treatments are good at organ involvement and neuropathy.

I thought you were DONE moving!!  OMG, your body does not need the extra stress right now. I hope this move is just short distance/local and you can get through this one relatively easily.   I assume you haven't heard about the MRI or CT??

So hoping you are right Anita re doctors taking symptoms seriously from now on. I think my journey to get to diagnosis has been such a rollercoaster one that I'm scared to expect anything good to come from it now. I haven't heard a word about my CT or MRI results from two weeks and one week ago respectively. But as I've said before - the NHS is very much teetering on the edge of crisis here and I think they would only alert me to something if it was immediately life threatening or required further investigations urgently. So it's reassuring to not have heard much - except of course that I feel that no further treatment options will be offered to me now if I have no organ involvement.

Only the vertigo/ disequilibrium, facial neuropathy/ pressure/ tingle and constipation and this weird pelvic pain are slowly worsening - the rest is much of a muchness really.

Re the latest house move - yikes it's a bit of a nightmare! We bought a small Victorian house early on this year and discovered many problems including wet rot, subsidence etc. We plan to live there when it becomes habitable and it's near Dundee where the teaching hospital is that I now attend. But meanwhile we still have a very warm, comfortable home on our old island where we have lived for 27 years. We have turned it into a self catering holiday let business but visitors only really go there in the summer months so we are going back there ourselves and I'll have to travel back down here once a month to pick up prescriptions, keep an eye on building work and attend hospital appointments. My husband's old job has been kept open for him so he is returning to this for six months until our new house is habitable. So we move out of our rented accommodation on Friday and all our furniture goes into storage.

Hope this explains. Dreading returning to wild winter island life as its a day's travel away. Same kind of distance as NYC to the bottom of Long Island plus a few more hours drive and a few hours on a ferry away.