Positive lip biopsy result

[MAT51]

Well it is very confusing but I had a parotid ultrasound today which was entirely normal. However the dental consultant and radiographer went to search out the results of my recent lip biopsy and apparently this meets the criteria for Sjogrens. He explained that all four salivary glands had shown over 50% lymphocytes - meaning I have Sjogren's despite not being badly afflicted with mouth dryness and only quite bad Sicca. So I'm guessing that this means that all my non length dependent small fibre neuropathy (everywhere including my face, scalp and all of peripheries) is certainly being caused by Sjogrens?  And so, finally this confirms what I have suspected for years - that I have an inflammatory neuropathy with Dysautonomia (heart palpitations, skewed sense of taste and smell, awful sweats but freezing cold, spikes in BP, chronic constipation, atrophy/ opposite with bizarre gynae features, disequilibrium) and that my high inflammation markers do relate to the neuro symptoms as I've long suspected.

My ANA (Nucleolar pattern) suggests  that I have Scleroderma rather than Sjogrens but I have raised immunoglobulins which suggest Sjogrens rather than Sclero - so how confused am I?!

Maybe at long last I'll get to try some more medications that will slow the neuropathy down and stave off organ related problems. I already have hypertension, a very large renal cyst and microscopic haematuria but these have all been shrugged off as unrelated problems by doctors to date. I'm hoping that with Hypothyrodism, seronegative RA and Sjogrens I might be able to try Mycophenolate.

[Jasper]

MAT .....

I am glad you finally have a diagnosis of Sjogren's after all of these years of trying to get answers. Hopefully now you will get the attention and treatment that you need. There are several possible treatments which may be of benefit to you. It is just a matter of trial and errot to see which ones work the best for you.

Have you seen a nephrologist about the microscopic hematuria? You could have interstitial nephritis or tubulointerstitial nephritis, which Sjogren's can cause.

http://cjasn.asnjournals.org/content/4/9/1423.full

[MAT51]

Thanks for this Jasper. No I haven't heard of these. Will study the link. I was under a hospital doctor last year (dramatic pancreatic reaction to Azathioprine/ Imuran) who was also a nephrologist but she thought my proteinura was due to the pancreatitis - later a sepsis. So hopefully the rheumatologist will arrange for me to see one. Mat

[SjoGirl]

Happy that you are gaining some very helpful information.

Another diagnosis a doctor might consider is Undifferentiated Connective Tissue Disease. UCTD is diagnosed in people who have symptoms of multiple AIs but not so many of any one disease that a doc can make a diagnosis. Treatment is much the same as for SJS, particularly given that there is no systemic treatment for SjS.

I have neuropathy with the cause not being clear. Fortunately it's not painful just makes me clumsier than I would like to be, particularly in my hands

[MAT51]

Thanks. The chap today said that the lip biopsy confirms primary SJS so I'm guessing my previous diagnosis of seronegative RA will go on the back burner now. But I think that this result means that it is differentiated as Sjogrens rather than UCTD? I maybe wrong about this though. I also think having an ANA Nucleolar pattern and raised IgG and IgA points quite specifically towards Scleroderma rather than UCTD. But again I could be wrong.

However I'm sincerely hoping that you are wrong about there being no disease modifying treatment for the systemic symptoms of Sjogrens. This is not what I've been led to believe. Someone I speak to on another forum was diagnosed with primary SJS and put straight onto Hydroxy with Rituxan. 

[Jasper]

There is a new guideline for treatment of Sjogren's Disease. I posted about it in a separate thread but will post some of it here and provide the link to the actual guidelines for you to read. The one thing I disagree with them on is that Rituxandoes definitely improves fatigue. I am on Rituxan and it dramatically improved my fatigue starting at about 4 weeks into treatment. It gave me back my life. All of the studies I read (about 5-6 studies) stated that Rituxan improved fatigue in Sjogren's Disease patients.

Medscape Medical News:   New Guidance on Managing Sjogren's Rheumatologic Problems, August 02, 2016

"The first US clinical practice guidelines for management of primary Sjogren's system were published online July 7 in Arthritis Care & Research and include a decision tree for the use of oral disease-modifying antirheumatic drugs (DMARDs) for inflammatory musculoskeletal (MSK) pain, guidance on patient self-care and exercise to reduce fatigue, and recommendations for uses of rituximab in Sjogren's. The Clinical Practice Guidelines Committee discouraged the use of tumor necrosis factor-α (TNFα) inhibitors for patients with Sjogren's who lack other indications for those agents, including patients with sicca symptoms. ..........

Hydroxychloroquine (HCQ) "remains the first line therapy for inflammatory musculoskeletal pain in Sjogren's ..........

The guideline provides a decision-tree table beginning with HCQ and progressing to methotrexate (MTX), then HCQ plus MTX, then short-term corticosteroids, then long-term (>1 month) corticosteroids.

The panel recommended that clinicians not use TNFα inhibitors to treat sicca symptoms in patients with primary Sjogren's, but consider them in patients who also have rheumatoid arthritis (RA) or related conditions. The authors also note there is "no evidence that the subset of RA patients with Sjogren's who have been treated with anti-TNF-α agents have an increased incidence of lymphoma. Therefore, this recommendation should not be interpreted to discourage use of TNF inhibitors in situations where there is overlap with RA or where TNFα inhibition therapy is indicated for the treatment of inflammatory arthritis."

The group recommended that clinicians consider rituximab for treating keratoconjunctivitis sicca in patients with primary Sjogren's inadequately controlled by conventional therapies including topical moisturizers, secretagogues, anti-inflammatory agents, immunomodulators, and punctual occlusion. They also recommended consideration of rituximab for xerostomia in patients with primary Sjogren's who have some residual salivary production and significant evidence of oral damage inadequately controlled by conventional therapies, including topical moisturizers and secretagogues.

The authors also recommend rituximab as a possible option for adults with primary Sjogren's and a variety of other systemic manifestations including vasculitis with or without cryoglobulinemia, severe parotid swelling, inflammatory arthritis, pulmonary disease, or peripheral neuropathy (especially mononeuritis multiplex). .........."

Full guidelines and discussion at:
https://www.sjogrens.org/files/research/RheumatologyCPG.pdf

[MAT51]

Gosh that's such an interesting read Jasper - many thanks. I actually live in Scotland where the decision on treatment guidelines for diseases is set by the Scottish Medicines Consortium (the Scottish equivalent of NICE). I did have a brief search for this yesterday but could only find stuff on RA. My guess is that it is much the same as the US guidelines you have posted here.

For my RA I have already tried Sulfasalazine (3 weeks then anaphylaxis), Methotrexate for two years (pills and injections) until it just made me too sick, Hydroxichloraquine ((18 months overlapping with MTX and on its own until it gave me anaphylaxis) and last year, Azathioprine/ Imuran which hospitalised me twice with pancreatitis! I have not been offered further modification/ immune suppression since - and have moved to a different part of Scotland now where they have started investigations again. Hence the lip biopsy and now the positive for primary Sjogrens. I seem to have a neuro type, very like MS. http://www.hopkinssjogrens.org/disease-information/sjogrens-
syndrome/neurologic-complications/

So as far as I see it my next treatment options are Mycophenolate - which is another DMARD that is increasingly used for people with Lupus, Vasculitis and Scleroderma - to avoid the ensuing renal involvement. It is expensive, but not nearly as expensive as Rituximab. It is better tolerated than the other DMARDs, has a good track record at treating the systemic side of these diseases but not helpful for moderating the inflammatory arthritis. My RA symptoms have been inactive for a long time now but are slowly coming back in both of my hands I think. So I may now meet the criteria for Rituximab but would rather be offered Mycophenolate first. If I have a diagnosis of UCTD as SjoGirl suggests, then I think I would still qualify for this medication - especially having previously failed to tolerate four conventional DMARDs. Hope this explains my situation. Many thanks.

[Tharrell]

Oh MAT, I am so, so glad things are finally getting nailed down for you! You came here on this board about the same time as me I believe. We all have been there with you! Now the doctors can shut up and treat you!

[MAT51]

Thanks Tharrell. Yes I remember us both joining at around the same time. I suppose this shows how long I've been confident that I do have Sjogren's as a primary disease. Hoping they will just get on with finding a treatment I can tolerate now but not expecting it because I've learnt the hard way that dashed expectations are worse than keeping them low! Mat x

[Judie P]

I had an interesting conversation today with my rheumy.  She is the doc for all Sjogren's patients because she came from the UCSF Sjogren's clinic in San Francisco.  I was talking to her about autonomic neuropathy because of my symptoms similar to yours.  It made sense what she said.  The digestive problems and constipation that I periodically get is not caused by Sjogrens.  I have had lesser issues with both since a young age.  Because I am older now (65), I continue to have these problems but the Sjogren's is making it all worse.  I also did not start with dry eyes or mouth.  In fact, mine was pretty good today.  Sometimes it gets dry but then stops.

When I walked into the hospital today, my blood pressure was 121/75.  Usually it is much higher.  My pulse was very low and the assistant was concerned.  She told her that it was normal for someone with Sjogren's to be in the 60s for pulse at times.  We took it aftewards and it was back to 85.  She went over my blood work since I was concerned about my BUN going from 12 to 21 in four months.  She told me not to worry.  All my other liver and kidney reports were good.   The rapid change was from being in a flair at the time I took the blood work and I was drier inside.

I think it can be very confusing at times, but we never know what our body is doing on any particular day.  I was lucky.  My ANA was positive and my SS-A was the highest it could go.  There was no confusion on whether or not I had SJS Primary.  I wish it could be that simple for all of you.

[MAT51]

Thanks Judy for sharing what the rheumy you spoke to said. After five years of living with a diagnosis of seronegative RA it does feel very gratifying to have a clear positive for both ANA (albeit signalling Scleroderma rather than SJS) and a definitive result showing primary Sjogren's from my lip biopsy. Sad to be so relieved and calm about this new result,  but I've been with many others in the shadowlands of misdiagnosed or undiagnosed for a long time now and I too feel almost blessed to get this clarity at last.

The thing is that my constipation and atrophy are both new and I'm 53 and had an early and uneventful menopause. So in my own case my instincts say that they are part of the Sjogren's autonomic issues rather than pre-existing symptoms made worse by the disease.

That said I did suffer from the more classic SJS symptoms when I was in my 20s and 30s - also symptoms corresponding with SLE and Hashimoto's - which I've been diagnosed with and treated for 13 years or so. I guess many of us are simply autoimmune babes. But post menopause my symptoms dramatically shifted away from chronic dryness, eczema, alopecia and endless rhinitis and conjunctivitis to my synovial joints. My inflammatory markers soared alongside swollen joints and my rheumatoid factor was a low but clear positive and rose over two years. Now I read that it was probably the primary Sjogren's that triggered the RA symptoms and then the polyneuropathy. So it's interesting to find another person for whom symptoms shift in this way.