Seronegative Sjogren, what's new?

[pattismith]

how is seronegative ss diagnosed - and if it is just diagnosed with a Schirmer test, why would it be ss in the first place? That is a thinking that makes no sense in a way?

sicca syndrome doesn't mean necessary Sjogren Syndrome.

SS diagnosis needs either positive Salivary gland biopsy or positive antibodies to anti-SS-A and anti-SS-B antigens.

Seropositive SS means antibodies to anti-SS-A and anti-SS-B antigens.
Seronegative SS means no antibodies to anti-SS-A or anti-SS-B antigens (but positive salivary gland biopsy).

Diagnosis and Management of Sjogren Syndrome - American Family Physician (aafp.org)

 

[bread.]

sicca syndrome doesn't mean necessary Sjogren Syndrome.

SS diagnosis needs either positive Salivary gland biopsy or positive antibodies to anti-SS-A and anti-SS-B antigens.

Seropositive SS means antibodies to anti-SS-A and anti-SS-B antigens.
Seronegative SS means no antibodies to anti-SS-A or anti-SS-B antigens (but positive salivary gland biopsy).

Diagnosis and Management of Sjogren Syndrome - American Family Physician (aafp.org)

positive gland biopsy - what is it showing what must it show?

 

[pattismith]

positive gland biopsy - what is it showing what must it show?

gland biopsy is positive when it shows lymphocytic sialaneditis:

"A biopsy is considered to be positive if histopathologic analysis shows a focus score of greater than or equal to 1 per 4 mm2 of tissue (a focus is defined as a cluster of 50 or more lymphocytes). With a focus score of greater than or equal to 1 as the cutoff, the sensitivity and specificity of labial salivary gland biopsy for the diagnosis of primary Sjögren's syndrome has been reported at 83.5% and 81.8%, respectively. "

I am seronegative, and my lip biopsy shows lymphocytic sialadenitis but grad doesn't reach level 1, so don't have SS diagnosis.

The thing is that I took corticosteroids the year before the biopsy, and I am B/B = O/O for MBL2 genotype/haplotype, so I have some doubts about the negative biopsy result...

Salivary Gland Biopsy - an overview | ScienceDirect Topics

 

[Rufous McKinney]

but the eyes and nose are dry every morning.

I'm sure I have some form of this but don't get tested, don't want any biopsies. There must be some other way.

The incredible dryness- is a major symptom here, cycles daily. So my eyes vary from pouring to dessicated. My nose is mostly totally dry as is my throat and lungs but not my mouth itself that much...I have a little saliva puddle there right now. Then it can dry from within the tissue itself..internal dryness (not lack of saliva).

I take zero medications that could cause dry mouth because that single side effect more or less results in my throat shutting down, cannot swallow etc.

 

[bread.]

gland biopsy is positive when it shows lymphocytic sialaneditis:

"A biopsy is considered to be positive if histopathologic analysis shows a focus score of greater than or equal to 1 per 4 mm2 of tissue (a focus is defined as a cluster of 50 or more lymphocytes). With a focus score of greater than or equal to 1 as the cutoff, the sensitivity and specificity of labial salivary gland biopsy for the diagnosis of primary Sjögren's syndrome has been reported at 83.5% and 81.8%, respectively. "

I am seronegative, and my lip biopsy shows lymphocytic sialadenitis but grad doesn't reach level 1, so don't have SS diagnosis.

The thing is that I took corticosteroids the year before the biopsy, and I am B/B = O/O for MBL2 genotype/haplotype, so I have some doubts about the negative biopsy result...

Salivary Gland Biopsy - an overview | ScienceDirect Topics

do you treat it in any systemic manner now?

 

[pattismith]

do you treat it in any systemic manner now?

no

 

[pattismith]

Abdominal symptoms during Sjogren’s syndrome: a pilot study | Advances in Rheumatology | Full Text (biomedcentral.com)
2021

result: .....
Regarding the pSS activity, in relation to European League Against Rheumatism (EULAR) Sjögren syndrome disease activity index score items, general and central nervous system involvement were associated with a high global symptom score
.....

Multivariate analysis showed a significant association between a high global symptom score and SSA seronegativity, gastroparesis, and ESSPRI score (p < 0.01 for each).

Abdominal symptoms during Sjogren’s syndrome: a pilot study (biomedcentral.com)

 

[Ljcha1892]

I have NLD SFN and Dysautonomia. My lip biopsy was 0.9 focal score so no SS diagnosis for me.

My sister has SS and more recently SLE. She is 10 years older than me and her antibodies only popped up last year but she’s been on off poorly for years with fatigue and other things but has never been as incapacitated as me. She’s fit and healthy and runs/plays soccer etc and is almost 50. However her eyes are so dry she has special drops made from her own blood, I can’t recall the name of this. (she’s in Australia- I am in U.K.)
I keep feeling that one day I may be antibody positive.

 

[bread.]

I have NLD SFN and Dysautonomia. My lip biopsy was 0.9 focal score so no SS diagnosis for me.

My sister has SS and more recently SLE. She is 10 years older than me and her antibodies only popped up last year but she’s been on off poorly for years with fatigue and other things but has never been as incapacitated as me. She’s fit and healthy and runs/plays soccer etc and is almost 50. However her eyes are so dry she has special drops made from her own blood, I can’t recall the name of this. (she’s in Australia- I am in U.K.)
I keep feeling that one day I may be antibody positive.

do you have found any treatment for yourself? how severe is your me/cfs?

 

[Ljcha1892]

I am not sure how to grade it. At one stage I was bedridden totally. That was first year. I had enormous cognitive issues, at that stage. It was a terrifying experience. The sfn developed rapidly during that time and was an overnight onset.

I have improved over the past 4 years in that I can pop out most days for a short while when rested and feeling stable enough to do so. Other days I stay home as I know going out is a bad idea.

I had an unplanned pregnancy in 201and had a son 17 months ago and pregnancy definitely improved my overall status. My sfn receded when pregnant but had relapsed since. My POTs is stable, I am not dizzy on standing most days but I am massively tachycardic from doing very little and physically my body just hurts, all the time:

Being a parent with this is very hard, I feel revolting most of the time and some days I do need help from family but mostly I manage ok, in my own unconventional way.

I have been unable to return to work as I just cannot stick to a schedule as this condition is so unpredictable and when I have tried small amounts of desk based admin work I crash for several days afterwards which is just not an option for me.

I have had a few courses of prednisone which did make me feel a bit better, but the side effects are rotten. Otherwise I just take pregabalin for pain and I’m just starting ivabradine again as my cardio wants to see if it improves my fatigue by treating my OI.

 

[Rufous McKinney]

Abdominal symptoms during Sjogren’s syndrome: a pilot study | Advances in Rheumatology | Full Text (biomedcentral.com)
2021

I am far more convinced than ever, I have this going on. No lip biopsy. My recent bouts of gastroperesis are tied to some form of autonomic dysfunction and worseneded ME.

My eyes are pouring liquid most of the time, unless they dry out entirely. Which happens occasionally. It comes on like a switch: in the late middle of the night, my eyes can focus and function. Then- well they start pouring liquid....it seems to be triggered by: something that cycles daily.

 

[pattismith]

I have NLD SFN and Dysautonomia. My lip biopsy was 0.9 focal score so no SS diagnosis for me.

My sister has SS and more recently SLE. She is 10 years older than me and her antibodies only popped up last year but she’s been on off poorly for years with fatigue and other things but has never been as incapacitated as me. She’s fit and healthy and runs/plays soccer etc and is almost 50. However her eyes are so dry she has special drops made from her own blood, I can’t recall the name of this. (she’s in Australia- I am in U.K.)
I keep feeling that one day I may be antibody positive.

are you MBL deficient?

Your Lip biopsy score doesn't seem far from the cutoff, so if you do it again at another time (well chosen), maybe result will be positive....

 

[Ljcha1892]

Sorry, whats MBL?

I won’t get another biopsy without paying for it, as the NHS has discharged me from their Sjögren’s rheumatology service on the basis of a negative biopsy. I asked another rheumatologist that I see on occasion if the biopsy being so close to the cutoff was significant and he said no, not really!

 

[halcyon]

I am seronegative, and my lip biopsy shows lymphocytic sialadenitis but grad doesn't reach level 1, so don't have SS diagnosis.

Out of curiosity, have you ever looked into/been worked up for IgG4-Related Disease?

I very recently, and suddenly, developed sicca symptoms to the point where I can't swallow food anymore, so it looks like I can be added to the list of ME with potential sicca syndrome or something like it.

The reason I ask is because I was looking into all this and found some indication that IgG4-Related Disease can essentially mimic Sjögren's syndrome (e.g. IgG4-related sialadenitis and Sjögren's syndrome; The immunobiology and clinical characteristics of IgG4 related diseases). I've personally never had any other indications of autoimmune disease, but I have had elevated IgG4 levels, and also started to develop some odd fibromas on my foot (which may or may not be related). Regardless I'm going to be doing all the Sjögren's bloodwork as well as IgG4 levels. I don't expect any satisfying answers though as I'm bedbound and unable to get any biopsies done.

 

[Gingergrrl]

I very recently, and suddenly, developed sicca symptoms to the point where I can't swallow food anymore, so it looks like I can be added to the list of ME with potential sicca syndrome or something like it.

Do you mean that you cannot swallow food b/c your throat is so dry or are you also having some kind of allergic reactions to food (or other issues)?

I've personally never had any other indications of autoimmune disease

I remember (from prior years) that you did not have autoimmune issues but have you been tested for any autoantibodies more recently? I was curious since many post-viral issues later flip into autoimmunity like what happened in my case.

Regardless I'm going to be doing all the Sjögren's bloodwork as well as IgG4 levels.

That is great

I don't expect any satisfying answers though as I'm bedbound and unable to get any biopsies done.

I am so sorry to hear that you are bedbound and often wondered throughout the years how you were doing and wish you the best

 

[halcyon]

Do you mean that you cannot swallow food b/c your throat is so dry or are you also having some kind of allergic reactions to food (or other issues)?

It seems, as far as I'm able to tell, to be due to dry mouth/throat. I can swallow water and Ensure without issues, it's just anything more viscous or solid than that and my swallow reflex doesn't properly kick in when I try to swallow and so I basically almost choke.

I remember (from prior years) that you did not have autoimmune issues but have you been tested for any autoantibodies more recently? I was curious since many post-viral issues later flip into autoimmunity like what happened in my case.

I haven't been able to access blood testing since 2019 due to being bedbound. I was finally able to find a mobile phlebotomy service so it looks like I should be able to get testing now. I am very curious to see if anything has flipped now at year 8 of ME, I definitely won't be surprised if it has given how common autoimmunity seems to be in ME.

 

[Gingergrrl]

It seems, as far as I'm able to tell, to be due to dry mouth/throat. I can swallow water and Ensure without issues, it's just anything more viscous or solid than that and my swallow reflex doesn't properly kick in when I try to swallow and so I basically almost choke.

I know that you can't physically access this now but in the future, do you have a doctor who can order a "swallow study"? It is often done in the hospital but I assume it could be done as an outpatient, too.

Also (just in case it is helpful), have you ever tried to drink "Orgain" in addition to Ensure to have more variety? The chocolate & mocha Orgain both actually taste really good and it's a very healthy product. But if you were having allergic reactions, it would not be good b/c it has so many different fruits, vegetables & ingredients in it.

I haven't been able to access blood testing since 2019 due to being bedbound. I was finally able to find a mobile phlebotomy service so it looks like I should be able to get testing now. I am very curious to see if anything has flipped now at year 8 of ME, I definitely won't be surprised if it has given how common autoimmunity seems to be in ME.

That is great that you found a mobile phlebotomy service and I used one in the past (many years ago) when I was sending blood to Germany for testing. They came to my home and drew the blood, prepared the sample, and even mailed it to the lab for me. I hope you will be able to get all of the tests that you need and it goes smoothly. Do you still see the same ME/CFS specialist (and please don't answer if you want to keep this private).