" Nobody much on this forum has sicca syndrome as far as I know, " = I think this is unfair to claim without measuring their tears and saliva, right?
Is there a connection between ME/CFS and Sjogren's Syndrome? Discussion and a poll about testing
- Thread starter TigerLilea
- Start date
" Nobody much on this forum has sicca syndrome as far as I know, " = I think this is unfair to claim without measuring their tears and saliva, right?
sorry but you're also gaslighting here Sjogrens' patients. No, Sjogrens fatigue is not deficiency of iron, I have enough friends with PEM who haven't left their homes for a DECADE, due to PEM, SFN, dysautonomia, and YES, they have positive SSA, salivary gland biopsy and salivary ultrasound (in a University hospital - not in 'private practice' which Johnathan Edwards speaks about - p.s. can I get a contact of these private physicians who give diagnoses by magic? asking for insurance purposes).
Jonathan Edwards
Senior Member (Voting Rights)
That's easy. Most people diagnosed with Sjogren's in private clinics probably do not have it. Even in my NHS clinic the majority of people who thought they had Sjogren's didn't. Some of them probably have ME/CFS. It might not sound so good for reimbursement and stuff though.
Neurological involvement in Sjogren's does occur but doesn't have much to do with the clinical picture of ME/CFS. Features of ME/CFS don't get called neurological involvement because so far we don't have clinical evidence that they are neurological, even if we suspect that.
Jonathan Edwards
Senior Member (Voting Rights)
You don't have to measure anything to know you have dry eyes. A positive test without symptoms is probably meaningless.
Utsikt
Senior Member (Voting Rights)
There’s not much of that in ME/CFS. If it was true that a «huge %» of ME/CFS cases actually was Sjögren’s or had comorbid Sjögren’s, don’t you think similar findings would have shown up in studies on ME/CFS by now?
Do you mean orthostatic intolerance? I’m not sure we know much about what causes that. Maybe I’ve missed some studies? Please share if you’re sitting on some convincing ones.
There are no signs of neuronal damage in ME/CFS so far, and certainly not on the scale of MS. DecodeME can be taken to imply neuronal involvement in ME/CFS, but that’s different from the kind of damage observed in MS.
How do you define PEM?
I’m asking because it frequently is described as simply a worsening of symptoms following exertion, but that’s far too broad because it would also involved exercise intolerance, DOMS, rapid fatigueability etc. Those are aspects of ME/CFS (probably primarily due to deconditioning), but they are not a part of PEM.
If you take PEM to include exercise intolerance, you could say that very many diseases have PEM.
We should look up how do ophthalmologists define dry eye, pretty sure it's by low tear on Schirmer and that even if you don't feel issues (like myself) you are advised to use eye drops, like I was, to prevent corneal damage.
OrganicChilli
Senior Member (Voting Rights)
And PEM isn't fatigue. If your friends have true PEM, they also have ME/CFS. If fatigue is their main issue, that's a Sjogren's thing I guess.
Sorry, but I find these nuances of PEM ridiculous because it's all arbitrary definitions that even 2 MECFS patients among themselves can't agree on and basically comes down to biological proof of what ME is, so once you have that, we can discuss the differences. Until then you're equally wrong as I am because you're making your own definitions (as a group).
But it did show up in studies.
I am not even sure what is not plausible here, the data says about 50% of large fiber neuropathy females had Sjogrens confirmed, high rates in NMOSD too, in SFN, POTS as well.
Isn't there confirmed SFN in a significant % of MECFS patients?
They have Sjogren's, giving symptoms of PEM, MECFS... as Sjogren's is higher in hierarchy and can cause many neuropsychiatric complications. They DONT have MECFS as a 'comorbidity'.
Why are you claiming that while Sjogren's is documented and known to cause very severe neurological conditions like NMOSD / paralysis, blindness, MS, motor neuropathies*... that it can't cause PEM? PEM is just a set of symptoms, anyway.
*perhaps because you're not aware of research in neurological autoimmune illnesses and how big % of those patients have Sjogren's too.
Utsikt
Senior Member (Voting Rights)
I think we’re just going to have to disagree here. Feel free to elaborate on your stance in the PEM factsheet thread if you feel like there’s anything seriously wrong with the definition outlined there.
Which studies? I feel like I’m repeating myself, but you keep dodging the question.
Where is this data from?
Where has that been confirmed?
Jonathan Edwards
Senior Member (Voting Rights)
And that'll be $100, many thanks?
I have spent my life in this business. I have looked after people with corneal damage from Sjogren's. I couldn't care less how an ophthalmologist wants to define dry eyes. I never met anyone without symptoms who came to harm. If they did then there should be a screening programme for all adults to have annual Schirmer tests. There comes a point when when needs to return to reality.
OrganicChilli
Senior Member (Voting Rights)
Higher in what hierarchy? Because we know more about it, it's a superior disease? You're not making any sense.
What even are Sjogren's giving symptoms of PEM? If they have PEM, they have ME/CFs whether or not they've been diagnosed with it.
What even are Sjogren's giving symptoms of PEM? If they have PEM, they have ME/CFs whether or not they've been diagnosed with it.
Jonathan Edwards
Senior Member (Voting Rights)
I am sorry if I sound a bit dismissive about people's pet theories but S4ME is such a good place for trying to raise the level of debate and evidence for ME/CFS science and if we degenerate into hand waving statements based on private physicians' proclamations we will slide back into the morass we have barely crawled out of.
Verity
Senior Member (Voting Rights)
I had started to suspect that patients with ME/CFS were somehow getting diagnosed with “neurosjogren’s” instead, much like some seem to get inappropriately diagnosed with hEDS or chronic Lyme. @Jonathan Edwards am I understanding correctly that Sjogren’s could be overdiagnosed even when antibody tests and biopsies are used?
Verity
Senior Member (Voting Rights)
I really agree. I’ve been getting a bit worried and frustrated with how often this happens on here lately.
This is 5+ years old, recently, there has been more research, especially with the formal inclusion of SFN on Sjogren's disease activity scales.
Neurologic involvement frequently preceded the diagnosis of SS
(81% of patients).
pubmed.ncbi.nlm.nih.gov
Neurological manifestations may precede the sicca symptoms in 40 to 93% of
the cases [8, 14]. As described by Mori et al. [11], 93% of patients were
diagnosed with pSS after neuropathy symptoms appeared.
Furthermore, CNS involvement may precede clinical diagnosis by many
years and may lead to an underestimation of other neurological and/or
systemic diseases [10].
Neurological onset often precedes by many years both the appearance of
systemic symptoms and the immunological diagnosis. Thus, pSS should
always be considered in patients with relatively nonspecific neurological
symptoms associated with sicca syndrome. MRI and neuropsychological
assessment are necessary for an early diagnosis, particularly when
neurological symptoms precede systemic involvement. Because of the great
heterogeneity of neurological involvement in pSS, neurological
complications should be the subject of large prospective cohort studies.
jmedicalcasereports.biomedcentral.com
Neurological symptoms preceded the diagnosis of SS in eight patients.
Conclusions
Peripheral neuropathies are frequent in SS patients. Neurologists should be aware of possible
autoimmune causes of neuropathies because clinical manifestations of neuropathy may
precede the development of other symptoms of the autoimmune disease.
Peripheral neuropathy may be the first symptom, but may also develop later in
the course of the disease.
In 8 (35%) of 23 PNS+ patients, neurological symptoms preceded the diagnosis
of pSS.
Some of the previous studies suggest that nervous system involvement can be the
presenting feature of SS, while others report that it is rather a late finding in the course
of the disease (Govoni et al., 1999; Grant et al., 1997; Griffin et al., 1990; Malinow
et al., 1986; Mori et al., 2005; Vrethem et al., 1990). Berkowitz et al. found that
neurological symptoms preceded the diagnosis of SS in 81% of patients (Berkowitz &
Samuels, 2014). In another study, conducted by Seeliger et al., a diagnosis of Sjögren's
syndrome was made in 44 of 184 (24%) patients with symptoms and signs of a severe
progressive symmetric or asymmetric peripheral neuropathy (Seeliger et al., 2019). In
our group, neurological symptoms preceded the diagnosis of pSS in 35% of patients.
This fact implies that these are patients who will first report to a neurologist.
In our cohort, neurological manifestations preceded the sicca symptoms in 72.1% of the
patients, which is consistent with the findings of other studies. 17–19
In 25-92% of patients affected by Sjögren’s syndrome, neurological symptoms may precede
the sicca syndrome.
“At least one-third of patients with pSS can present with extraglandular involvement, with
neurological dysfunction being one of the most frequent conditions… In 25–60% of cases,
neurological symptoms are the first clinical manifestation and can precede the diagnosis of pSS
on an average of two years,” the scientists wrote.
Neurological manifestations may precede. SICCA symptoms, so all patients with
polyneuropathy would be tested for Sjogren's syndrome.
However, neurological manifestations occur in 10 to 60% of patients with Sjögren's
syndrome and can often precede classic sicca symptoms in Sjögren's syndrome in some
cases up to several years. Rarely, cranial neuropathy can be the initial presentation. Thus, it
is important to consider screening for Sjögren's syndrome in the evaluation of pediatric
patients with new onset of isolated cranial neuropathy even in the absence of classic sicca
symptoms.
Q: Can you explain this study in plain English?
A: Sure. Dysautonomia International collaborated with the neurologists at South Shore Neurologic
Associates in New York and Sjögren’s researchers at SUNY Buffalo. We looked at the records of all
of South Shore Neurologic Associates’ patients over the past year who reported either dry eye or
dry mouth symptoms, plus some kind of other autonomic problem, who didn’t have an
identifiable cause (meaning their dysautonomia was “idiopathic”), and who didn’t have the SS-A
and SS-B antibodies doctors common rely upon to diagnose Sjögren’s. Out of 95 idiopathic
dysautonomia patients included the study, we found that 41% of them had one or more novel
early Sjögren’s antibodies (salivary protein-1 [SP-1], parotid secretory protein [PSP], and/or
carbonic anhydrase-6 [CA-6]). Then we looked at the symptoms found in the antibody positive
patents compared to the symptoms in the antibody negative patients. They essentially had the
same symptom profiles, but the antibody positive patients were more likely to have constipation.
Q: Is Sjögren’s common in dysautonomia patients?
A: Sjögren’s is the second most common cause of autonomic neuropathy, after diabetes, and has
been associated with postural orthostatic tachycardia syndrome, orthostatic hypotension,
orthostatic intolerance, autoimmune autonomic ganglionopathy, gastroparesis, and other forms
of dysautonomia. In fact, the dry eye that Sjögren’s is well known for is a symptom of
dysautonomia, since the tear glands are controlled by the autonomic nervous system. 1 in 10
people who have dry eye have Sjögren’s syndrome. Sjögren’s impacts 4 million Americans, but 3
million of them are undiagnosed.
Q: Isn’t Sjögren’s an older woman’s disease?
A: The stereotypical Sjögren’s patients is a Caucasian woman over age 40. However,
Sjögren’s can occur in any race or ethnicity, and 10% of patients are male. While Sjögren’s
is not as common in children as it is in adults, it can occur at any age. The youngest novel
Sjögren’s antibody positive patient in our study was 13. Younger Sjögren’s patients tend
to present with different symptoms than older patients, with more neurological
symptoms and less dryness. The dryness usually develops slowly over time as the
disease progresses.
The current diagnostic criteria for Sjögren’s were developed based on studies of older
patients who have advanced/severe dryness associated with their Sjögren’s. Many experts
now agree that the current diagnostic criteria are not catching patients who are at an
earlier stage of the disease, who tend to be younger and have less severe dryness, when
you may actually be able to prevent some of the long term damage from occurring. People
diagnosed with Sjögren’s in their 50s have probably been dealing with it for 20-30 years
before they were “sick enough” to get diagnosed.
Neurologic Complications
A PRIMER ON THE NEUROLOGICAL COMPLICATIONS OF SJÖGREN’S SYNDROME
www.hopkinssjogrens.org
complications/
Neuro-Sjögren: Peripheral Neuropathy With Limb
Weakness in Sjögren's Syndrome
www.ncbi.nlm.nih.gov
Small Fiber Neuropathy in Sjogren's Syndrome: A Review
review
Immunoglobulin Injections May be Effective for Peripheral Nerve Damage in Sjogren’s
Patients, Study Says
Neurologic involvement frequently preceded the diagnosis of SS
(81% of patients).
Neurologic manifestations in primary Sjögren syndrome: a study of 82 patients - PubMed
Neurologic involvement occurs in approximately 20% of patients with primary Sjögren syndrome (SS). However, the diagnosis of SS with neurologic involvement is sometimes difficult, and central nervous system (CNS) manifestations have been described rarely. We conducted the current study to...
pubmed.ncbi.nlm.nih.gov
Neurological manifestations may precede the sicca symptoms in 40 to 93% of
the cases [8, 14]. As described by Mori et al. [11], 93% of patients were
diagnosed with pSS after neuropathy symptoms appeared.
Furthermore, CNS involvement may precede clinical diagnosis by many
years and may lead to an underestimation of other neurological and/or
systemic diseases [10].
Neurological onset often precedes by many years both the appearance of
systemic symptoms and the immunological diagnosis. Thus, pSS should
always be considered in patients with relatively nonspecific neurological
symptoms associated with sicca syndrome. MRI and neuropsychological
assessment are necessary for an early diagnosis, particularly when
neurological symptoms precede systemic involvement. Because of the great
heterogeneity of neurological involvement in pSS, neurological
complications should be the subject of large prospective cohort studies.
Peripheral and central nervous system involvement in a patient with primary Sjögren’s syndrome: a case report - Journal of Medical Case Reports
Background Primary Sjögren’s syndrome is the second most common rheumatological disorder after rheumatoid arthritis. It typically presents as xerophthalmia and xerostomia in postmenopausal women. Involvement of the central nervous system has been recognized, although its pathogenesis and...
jmedicalcasereports.biomedcentral.com
Neurological symptoms preceded the diagnosis of SS in eight patients.
Conclusions
Peripheral neuropathies are frequent in SS patients. Neurologists should be aware of possible
autoimmune causes of neuropathies because clinical manifestations of neuropathy may
precede the development of other symptoms of the autoimmune disease.
Peripheral neuropathy may be the first symptom, but may also develop later in
the course of the disease.
In 8 (35%) of 23 PNS+ patients, neurological symptoms preceded the diagnosis
of pSS.
Some of the previous studies suggest that nervous system involvement can be the
presenting feature of SS, while others report that it is rather a late finding in the course
of the disease (Govoni et al., 1999; Grant et al., 1997; Griffin et al., 1990; Malinow
et al., 1986; Mori et al., 2005; Vrethem et al., 1990). Berkowitz et al. found that
neurological symptoms preceded the diagnosis of SS in 81% of patients (Berkowitz &
Samuels, 2014). In another study, conducted by Seeliger et al., a diagnosis of Sjögren's
syndrome was made in 44 of 184 (24%) patients with symptoms and signs of a severe
progressive symmetric or asymmetric peripheral neuropathy (Seeliger et al., 2019). In
our group, neurological symptoms preceded the diagnosis of pSS in 35% of patients.
This fact implies that these are patients who will first report to a neurologist.
In our cohort, neurological manifestations preceded the sicca symptoms in 72.1% of the
patients, which is consistent with the findings of other studies. 17–19
In 25-92% of patients affected by Sjögren’s syndrome, neurological symptoms may precede
the sicca syndrome.
“At least one-third of patients with pSS can present with extraglandular involvement, with
neurological dysfunction being one of the most frequent conditions… In 25–60% of cases,
neurological symptoms are the first clinical manifestation and can precede the diagnosis of pSS
on an average of two years,” the scientists wrote.
Neurological manifestations may precede. SICCA symptoms, so all patients with
polyneuropathy would be tested for Sjogren's syndrome.
However, neurological manifestations occur in 10 to 60% of patients with Sjögren's
syndrome and can often precede classic sicca symptoms in Sjögren's syndrome in some
cases up to several years. Rarely, cranial neuropathy can be the initial presentation. Thus, it
is important to consider screening for Sjögren's syndrome in the evaluation of pediatric
patients with new onset of isolated cranial neuropathy even in the absence of classic sicca
symptoms.
Q: Can you explain this study in plain English?
A: Sure. Dysautonomia International collaborated with the neurologists at South Shore Neurologic
Associates in New York and Sjögren’s researchers at SUNY Buffalo. We looked at the records of all
of South Shore Neurologic Associates’ patients over the past year who reported either dry eye or
dry mouth symptoms, plus some kind of other autonomic problem, who didn’t have an
identifiable cause (meaning their dysautonomia was “idiopathic”), and who didn’t have the SS-A
and SS-B antibodies doctors common rely upon to diagnose Sjögren’s. Out of 95 idiopathic
dysautonomia patients included the study, we found that 41% of them had one or more novel
early Sjögren’s antibodies (salivary protein-1 [SP-1], parotid secretory protein [PSP], and/or
carbonic anhydrase-6 [CA-6]). Then we looked at the symptoms found in the antibody positive
patents compared to the symptoms in the antibody negative patients. They essentially had the
same symptom profiles, but the antibody positive patients were more likely to have constipation.
Q: Is Sjögren’s common in dysautonomia patients?
A: Sjögren’s is the second most common cause of autonomic neuropathy, after diabetes, and has
been associated with postural orthostatic tachycardia syndrome, orthostatic hypotension,
orthostatic intolerance, autoimmune autonomic ganglionopathy, gastroparesis, and other forms
of dysautonomia. In fact, the dry eye that Sjögren’s is well known for is a symptom of
dysautonomia, since the tear glands are controlled by the autonomic nervous system. 1 in 10
people who have dry eye have Sjögren’s syndrome. Sjögren’s impacts 4 million Americans, but 3
million of them are undiagnosed.
Q: Isn’t Sjögren’s an older woman’s disease?
A: The stereotypical Sjögren’s patients is a Caucasian woman over age 40. However,
Sjögren’s can occur in any race or ethnicity, and 10% of patients are male. While Sjögren’s
is not as common in children as it is in adults, it can occur at any age. The youngest novel
Sjögren’s antibody positive patient in our study was 13. Younger Sjögren’s patients tend
to present with different symptoms than older patients, with more neurological
symptoms and less dryness. The dryness usually develops slowly over time as the
disease progresses.
The current diagnostic criteria for Sjögren’s were developed based on studies of older
patients who have advanced/severe dryness associated with their Sjögren’s. Many experts
now agree that the current diagnostic criteria are not catching patients who are at an
earlier stage of the disease, who tend to be younger and have less severe dryness, when
you may actually be able to prevent some of the long term damage from occurring. People
diagnosed with Sjögren’s in their 50s have probably been dealing with it for 20-30 years
before they were “sick enough” to get diagnosed.
Neurologic Complications
A PRIMER ON THE NEUROLOGICAL COMPLICATIONS OF SJÖGREN’S SYNDROME
Neurologic Complications : Johns Hopkins Sjögren’s Center
Neurological complications may affect 10- 20% of those with Sjögren’s disease and range from cognitive difficulties to burning toes and feet. Introduction to the Nervous System The nervous system is […]
www.hopkinssjogrens.org
Neuro-Sjögren: Peripheral Neuropathy With Limb
Weakness in Sjögren's Syndrome
Neuro-Sjögren: Peripheral Neuropathy With Limb Weakness in Sjögren's Syndrome - PMC
Objective: Sjögren's syndrome is a heterogeneous inflammatory disorder frequently involving peripheral nerves with a wide spectrum of sensory modalities and distribution patterns. The objective of this cross-sectional study was to determine ...
www.ncbi.nlm.nih.gov
review
Immunoglobulin Injections May be Effective for Peripheral Nerve Damage in Sjogren’s
Patients, Study Says
Not superior. But MECFS is a set of symptoms? Sjogrens is defined from immunological profile and is multi-systemic, check the formal definition and disease activity scales. ONE part of symptoms is attack to the nervous system, it is known to cause many autoantibodies, B cells hyperactivity - thus high IgG. Some systems like peripheral nervous system is attacked more often.
So yes: from defining the disease pathology it is higher because Sjogrens can, in theory at least, cause MECFS symptoms, while MECFS can't cause Sjogrens. We also know much more about what is Sjogrens (immunologically) vs what is MECFS (for now: set of symptoms).
It is evident that people posting on this thread are not aware that Sjogren's is frequently causing severe neurological illnesses like NMOSD and CIDP, that can make one paralysed and blind, so I think the relying on 'I feel Sjogrens couldnt possibly cause something as bad as PEM' is just relying on prejudice, without information.
Verity
Senior Member (Voting Rights)
But what does neurological involvement in Sjogren’s actually look like? Where’s the evidence it looks like ME? It’s not enough to say Sjogren’s can have neuro involement, and ME might be neurological, so Sjogren’s can look like ME.
The quotes posted talk about dysautonomia and neuropathy, but neuropathy is not ME, and dysautonomia alone is not ME even though it often comes with it.
The quotes posted talk about dysautonomia and neuropathy, but neuropathy is not ME, and dysautonomia alone is not ME even though it often comes with it.
I’m homebound due to Sjogrens disease with post-exertional malaise (PEM) and small-fiber neuropathy (SFN). I’m SSA-positive and ANA-negative. My lip and minor salivary gland biopsies are positive. Aside from ME/CFS and SFN, I don’t have other symptoms, and I don’t really notice dryness because the PEM/ME-CFS symptoms are worse.