Blood test negative but is that even enough?should I have a lip biopsy!

I've tried Sulfasalazine, Methotrexate, Hydroxichloraquine, Azathioprine and now Mycophenolate. I had severe reactions to all apart from Mycophenolate which I'm doing well on. It depends how severely you are affected. My Sjogren's affects my peripheral and autonomic nervous system and my liver and kidneys so it is taken very seriously now despite being serum negative. My secondary Raynauds is problematic and my ANA pattern points more to Scleroderma (which I don't yet have). And yet, it wasn't for the lip biopsy result, I would still be untreated. 

Hi Just wondering about the ana pattern pointing to scleroderma?

 

My ANA was necleolar pattern 1:320 but I don't have Scleroderma yet, although I do have secondary Raynauds. I've had normal skin biopsy over two years ago and normal nerve conduction studies/ EMG etc but my neurologist is happy to confirm I do have SFN anyway and it's definitely progressed. She calls the autonomic dysfunction Ganglionopathy and is happy to presume this too because of my extremely positive lip biopsy results. 

Has the dr done any tests re to the peripheral neuropathy, muscle weakness and difficulty urinating? Have you got any saddle numbness?

Sorry about the questions.  xx

Yes thanks for your response I am going to push for a lip biopsy ! Hopefully I get some answers! Since I'm really starting to suffer now

Yes, he did a special muscle biopsy on my legs which were normal, but the neuropathy was just beginning. He sent ne to urologist who suggested physical therapy to learn how to relax bladder muscles. ( I passed on that) otherwise she was no hep, and he increased my nerontin to double and mirapex to double. I try not to take all that Mirapex because I feel dizzy in the morning if I take it all.  My rheumy increased my Cellcept or mycophenolate substantially to try and stop this. As I type this my left foot , leg and hip are killings me..so hard to get exercise when you hurt so much.

I agree. That's why I've so far avoided taking DMARDs or immunosuppressants. I'd rather manage my symptoms conservatively until such time as I need the big guns. And as I'm 73 and already 22 years on from the start of my symptoms (though, typically, only 12 years on from diagnosis!) I'm hoping that day won't come. That's also why I refused a lip biopsy when it was offered 10 years ago. The procedure isn't entirely without the risk of irreversible peripheral nerve damage, the results can be equivocal, and I figured there was no point going through it if I wasn't going to take any of the drugs on offer anyway.

Admittedly I've been lucky. I've had no serious organ damage as far as I know, apart from the inevitable attack on my thyroid, common to many autoimmune diseases, and, more recently, the first signs of inflammatory damage to my lungs. The thyroid deficiency is easily corrected by taking thyroxine, and use of a steroid inhaler for the past year or so has dramatically improved my lung capacity. The tendinitis is a real pain at the moment (in all senses!) particularly as it's now spread from my arms to one leg as well. However, I find I can keep it under some kind of control with cold compresses, regular gentle exercise and an occasional dose of ibuprofen if it's keeping me awake at night. The Raynaud's comes and goes in my left hand, but is only at nuisance levels, ditto the peripheral neuropathy in my hands and feet. I can also keep joint pain at bay for the most part by taking fish oil and glucosamine. I know the latter has been largely discredited but my GP says no harm in continuing. Oh, and I've just discovered some marvellous new OTC eye drops that are working wonders on the dry eyes!

Obviously those who have developed the more life-threatening consequences of Sjogren's (and especially of the much more serious scleroderma) will need more powerful treatments. I fell on my feet after a house move four years ago, when I found a new GP who seems to know more about autoimmune conditions than the rheumatologist I saw - and most others I've heard about. I've discussed this at length with him and he says that as long as I treat any newly-emerging symptoms as they arise (e.g. the recent lung inflammation) he sees no advantage in going on to more aggressive drugs.

Wow you seem very bright! I have a great RA Doc. Extremly thourough, maybe too thorough .I'm doing acuounture for some issues i have  And praying for the best.  They sat me down and explained its a manging symptoms kind of thing until you cant manage them and many can. It functions in degrees like anything else. I use refresh and systeane drops if you have better ones please feel free to message me. My eye dic said he is watching that as i have eye issues and if needed would give me restaisi.

 

Lily I take issue with you that Scleroderma is a much more serious disease. All these diseases are equally potentially serious if you have them badly. For some there is crossover and I think Sjögren's can me far more destructive than you realise - a bit of numbness here or there when you are in your 70s is one thing but if you are in your 30s or even, like me, in your 50s and have severe and painful neuropathy or get Lymphoma then there are years ahead of dealing with a multisystem body-wide autoimmune disease - same as Lupus, RA and Scleroderma. I'm really keen for this attitude of Sjögren's being a less serious disease stopping now. Some have mild RA, Lupus and Scleroderma and some have severe Sjögren's.

Well said mam - Sjogrens is a very serious disease has an elevated death rate reduced life expectancy of 50% post onset increased lyphoma risk and is life destroying for some like me who have is severely. It is a systemic disease and NOT JUST DRY eyes and mouth although god knows they are debilitating enough . All AI have ranges of patient experinces and in terms of "seriousness actaully SLE and SS are closely related and 2 of the more "dangerous" diseases as they impact the whole body and neither have effective treatments and both often lead to other AI like RA

Hi Tumtum, I'm coming back to this one late.

If you read any reputable medical site, you'll see that statistically scleroderma is the most serious disease of the three in this connective tissue autoimmune group. It has by far the highest death rate. (I still retain memories of nursing a woman in her late 30s dying a horrible death from scleroderma 50 years ago.)

According to most medical sites, Sjogren's doesn't statistically shorten life at all. For example, a 2004 NCBI concluded that: "No increased all-cause mortality could be detected for patients with primary SS compared with the general population." This study did, however, go on to mention that there was an increased mortality rate for SS sufferers who went on to develop non-Hodgkins lymphoma.

I believe that 50% increase in the rate of lymphoma is also being reviewed downwards at the moment. When I received my SS diagnosis 12 years ago, my doctor told me the risk of getting NHL was a whopping 66 TIMES (not percent) the risk compared with non-AI sufferers! I was offered two-yearly MRIs, covered by the State insurance, at the time, as the risk was considered so high. In the event, I decided not to take up the offer, preferring to remain watchful. But whether the risk is 50% or 3,300% higher, it's worth remembering the occurrence of NHL in the general population is not all that high anyway. You can scare yourself unduly with statistics.

It's important to bear in mind that all these analyses are made on a statistical basis. They're not saying that Sjogren's is never fatal or life-changing for everyone, nor are they saying that everyone with scleroderma is going to have a bad time.

The fact remains that most SS sufferers don't become seriously ill, and I think it's important to stress that to avoid worrying new sufferers unduly. Sites like this one tend to attract those most seriously affected - for quite understandable reasons, as you're the ones who need the most support. Unfortunately, this can skew the overall position for new sufferers, leading them to fear that they're inescapably heading for a lifetime of suffering and disability, which - statistically speaking - will not be the case for most of them.

Those of you who are suffering badly have my full sympathy. But the fact remains that even if you are in the majority on these boards (which I would estimate not to be the case) you're not in the majority among SS sufferers in general, most of whom never go near a support site. My aim has never been to minimise your sufferings but merely to put the other side of the argument: many Sjogren's sufferers do not become seriously disabled.

Lily65668 - my interest here is in speaking up for those of us who have full blown Sjögren's disease. For those with Sicca Syndrome - this is just a really unpleasant condition which, if treated topically, may well never progress. There are people who are newly diagnosed with Scleroderma who may read this too and I wouldn't want them to think that it is always severe and often fatal. All these AI diseases come in degrees of severity and it is possible to have Scleroderma mildly. 

I know that many think they have Sjögren's because of dry eyes and mouth - and some do actually have seropositive Sjögren's mildly (i.e not affecting organs or peripheral or autonomic nervous system). This I accept of course.

However there is very little that has been written about Sjögren's on even the most reputable sites, that doesn't need reviewing now. Part of the problem is historic it seems, the disease was only identified by Henrik Sjögren in 1933 and in terms of medical history this is quite recent so there's a lot of catching up for the medical establishment to do.

I have known two people who have died from Scleroderma related pulmanory embolisms. I have also known of several people amongst my autoimmune friends with Sjögren's who have died from related co-morbitities. The full blown version of our disease is equally serious to all the others, including Scleroderma. And of course Scleroderma is a much rarer disease than RA, Sjogren's or Lupus and, in a sense, it's a syndrome too and if you have mild CREST or the limited version, or if your systemic Scleroderma is caught and treated very early on it sometimes burns itself out. Therefore I stand by what I say. You can a take athlete like Venus Williams who does brilliantly in her field despite Sjogren's. You can take a great artist like Paul Klee who painted and achieved a great deal despite his Scleroderma. But please let us stop pitting one horrible disease against another - as if there was a hierarchy within the spectrum of rheumatic diseases. Who actually knows how many have died from Sjogren's since it so often goes unrecognised and undiagnosed?! All these autoimmune diseases affect each of us differently and speaking for myself, I want my primary Sjögren's to be taken as seriously by my medical team as it already is, and by societyat large as it very much is not!!

There is no such yjing as sicca sydrome and SS is a systemic disease and sicca is one of the most debilitating sympoms is life destroying and is systemic not "just eyrs and mouth" The increased mortality of SS is such that average life expectancy post onset  is 50% that of "normal" people. Sero + and - ad lip biopsies are ,eaningless as 50% plus are sero -ve and 10-20% lip biopsy -ve. The whole classificatio of SS by medicine is b******s outdated inaccurate useless and Venis Willaims doesn't have SS watever anyone says if she did she couldn't play elite tennis!

Actually I'm inclined to agree with you. 

Although I use the term Sicca to describe one of many nasty aspects of Sjögren's. People with other diseases and on many medications can have sicca without having Sjogren's. If you have terrible dry eyes and mouth its foul but if you have this plus kidney, lung, liver or brain involvement then it's even worse is what I meant. Either way I'm heartily sick of people minimising Sjögren's. I don't know what I think about Venus Williams - half inspired, half exasperated!