Severe difficulties with eating in ME/CFS
- Thread starter Jonathan Edwards
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I have asked this question before in a different topic (devoted to a case study about AAG), but would like to raise it here as well: why are ME/CFS patients not routinely tested for autoimmune autonomic ganglionopathy? This condition implies severe dysautonomia, abnormalities in pupillometry, sweating abnormalities and severe dysfunction in GI motility. It is regarded as rare, but not that many doctors have heard about this condition in order to refer patients to a knowledgeable neurologist. The blood test for ganglionic ACHR receptor antibodies is positive in 50% of the cases. The test is included in the Mayo dysautonomia blood panel.
Jonathan Edwards
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I don't think there is any suggestion that people with ME/CFS have these symptoms. As Nightsong has pointed out, the picture of 'POTS' is not actually dysautonomia in this sense. In POTS the autonomic nervous system seems to be working - in responding to standing with tachycardia. My memory is that people with dysautonomia often have uncontrollable diarrhoea, which is not a feature of ME/CFS. And so on.
Thank you very much for pointing this out.
Yes, I do now understand that this blood pressure and heart rate orthostatic response is preserved in patients with POTS and/or ME/CFS. It’s other components of autonomic failure (severe GI dysmotility in the form of severe constipation & early satiety) in people with AAG which made me interested in this condition.
Jonathan Edwards
Senior Member (Voting Rights)
That sounds a bit like Hirschsprung's disease with ganglionic agenesis (rather than diarrhoea in the Shy-Drager picture). Clearly this is a complicated field. I think the simple point is that people with ME/CFS don't really have generalised dysautonomia - of whatever sort.
Formerhuman
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Do some patients with Sjogren's have dysautonomia?
I think lots of us have symptoms like this that aren’t pots. And meet most of these in the general bug then things like the pupils or the sweating might be a different pattern etc
But I’m guessing there is something technical I’m missing that makes it not dysautonomia?
Jonathan Edwards
Senior Member (Voting Rights)
I have not heard of any link betwen Sjogren's and dysautonomia but 'Sjogren's' is treated by some as one of those categories that can spread out in to almost anything. I think it becomes largely meaningless at that point.
Formerhuman
Established Member (Voting Rights)
That's what I see in a popular textbook of rheumatology FIRESTEIN & KELLEY’S TEXTBOOK OF RHEUMATOLOGY,
TWELFTH EDITION, Chapter 74 "Sjogren's syndrome" by E. WILLIAM ST. CLAIR AND DAVID L. LEVERENZ, page 1287:
Peripheral nervous system involvement is among the most
common of the extraglandular features of primary Sjögren’s
syndrome. In a cross-sectional study, peripheral neuropathy was
diagnosed in 17 (27%) of 62 patients with the primary type on
the basis of a conventional neurologic examination.126 However,
only 34 (55%) of the patients with primary Sjogren’s syndrome
in this group had abnormal nerve conduction velocity studies,
including 19 (31%) with a motor neuropathy, 8 (13%) with a
sensory neuropathy, and 7 (11%) with a sensorimotor neuropathy.
In this study, two patients with normal nerve conduction
velocity studies were diagnosed with a small-fiber neuropathy,
which is characterized by the loss of nerve fibers less than 7
micrometers, and thus cannot be assessed by conventional nerve
conduction studies.127 Small-fiber neuropathies are increasingly
recognized in a large proportion of patients with primary
Sjögren’s syndrome, although the true prevalence is unknown.
Typically, small-fiber neuropathies present with painful neuropathic
symptoms and may be associated with autonomic dysfunction,
such as vasomotor symptoms, hyperhidrosis, and
orthostasis.127
Another example is cross-sectional study of a UK cohort of 317 patients with PSS (https://doi.org/10.1136/annrheumdis-2011-201009)
Methods
Multicentre, prospective, cross-sectional study of a UK cohort of 317 patients with clinically well-characterised PSS. Symptoms of autonomic dysfunction were assessed using a validated instrument, the Composite Autonomic Symptom Scale (COMPASS). The data were compared with an age- and sex-matched cohort of 317 community controls. The relationships between symptoms of dysautonomia and various clinical features of PSS were analysed using regression analysis.Results
COMPASS scores were significantly higher in patients with PSS than in age- and sex-matched community controls (median (IQR) 35.5 (20.9–46.0) vs 14.8 (4.4–30.2), p<0.0001). Nearly 55% of patients (vs 20% of community controls, p<0.0001) had a COMPASS score >32.5, a cut-off value indicative of autonomic dysfunction. Furthermore, the COMPASS total score correlated independently with EULAR Sjögren's Syndrome Patient Reported Index (a composite measure of the overall burden of symptoms experienced by patients with PSS) (β=0.38, p<0.001) and disease activity measured using the EULAR Sjögren's Syndrome Disease Activity Index (β=0.13, p<0.009).I haven't dug into the question, but I have heard from patients and Sjögren's advocates that autonomic dysfunction is disabling and often dismissed by physicians and ignored in clinical trials.
In your recent interview with David Tuller, you said about the dysregulation of the autonomic nervous system: "It is all a bit doubtful."
If I may ask, how would you explain this clinical entity when during a mild infection a healthy person develops an excessive orthostatic rise in heart rate — say, 70 bpm before infection and 130 bpm after — and this becomes chronic? How would you explain this, given that mechanical factors, "small heart," and deconditioning are clearly not the case, and as you mentioned in the interview, the HPA axis seems to be intact among patients?
Hmm, seems like the most recent (2025) British society for rheumatology guideline on management of Sjögren’s disease also mentions various peripheral neuropathies as one of the features of systemic involvement.
Jonathan Edwards
Senior Member (Voting Rights)
That section is typically wooly and without objective figures. Unfortunately, we live in an age in which building clinical and research empires on this sort of waffle takes precedence over any hard data. I was recently talking to an expert on peripheral neuropathy who confirmed that tests for small fibre neuropathy are still largely meaningless. And 'may be associated with autonomic dysfunction' is about as vague as you can get. Special Sjogren's clinics are totally unrepresentative of the Sjogren's population as a whole.
Bascially it provides no factual evidence of a link and these days physicians will tend to suggest any link that fits with current fashion.
Jonathan Edwards
Senior Member (Voting Rights)
There must be a mechanism involving adrenergic nerves to the heart but calling this 'dysautonomia' doesn't help anybody in my view. Dysautonomia ought to mean some failure of the autonomic system itself. I think it much more likely that after an infection the autonomic nervous system responds to abnormal signals that arise outside it. If the problem is in a hypothalamic origin of the sympathetic drive that is one thing. If it is irritability of pacemaker cells in the heart it is another. It doesn't help to bundle them together.
For a period of about five years I used to wake up most mornings with a pulse rate of 150. It sometimes occurred in the middle of the night. It would last about five minutes. I consulted a cardiologist who thought I was 'functional'. I tried a beta blocker but that was horrible. After five years it went away completely. The cardiologist was not in the slightest bothered that he could not explain it. I think there are a lot of things like this we do not understand. I think they are better described in their own right rather than using these vague terms like dysautonomia which nobody actually knows the meaning of.
Formerhuman
Established Member (Voting Rights)
I think the example of pacemaker cell irritability has no connection with reality. I am not aware of any clinical examples of pathological pacemaker cell irritability causing orthostatic tachycardia. Most clinical examples of OI are seen in neuroimmune or neurodegenerative disorders such as LEMS, MSA, Sjögren's, diabetes, and Parkinson's disease. My view is that blood pressure and heart rate are mainly orchestrated by the autonomic nervous system and the endocrine system. And given the absence of clear HPA pathology, I would argue that dysregulation of the autonomic nervous system is far from doubtful.
Jonathan Edwards
Senior Member (Voting Rights)
But that does not mean that all are. People with ME/CFS do not have generalised features of autonomic failure as seen in these other conditions. So it is better not to lump them all together.