Promoting optimal care for Sjogren's, a common, serious, systemic disease.
AUTONOMIC DISORDERS / POTS
(Evaluation & Management)
Key Concepts
Do you have severe fatigue, lightheadedness, palpitations, difficulty concentrating (brain fog), nausea, or problems regulating your temperature? If so, you might have dysautonomia.
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Autonomic disorders (dysautonomia) such as postural orthostatic tachycardia syndrome (POTS) are common in Sjogren’s, but often remain undiagnosed (50, 151, pp. 164-167).
Learn more about the autonomic nervous system and dysautonomia on this page.
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Most clinicians have no dysautonomia training. Autonomic disorders are not detected by a routine neurological evaluation. They are diagnosed using special tests, such as the standing test, that clinicians may not be familiar with.
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Because autonomic disorders cause symptoms in multiple seemingly unconnected parts of the body, clinicians sometimes default to psychological explanations. When this happens, patients are deprived of diagnosis and treatment of a neurological condition.
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If you have dysautonomia symptoms, it is important to come to your clinic visit prepared. This page will help you do that. While this may seem like a lot of work, my POTS diagnosis story illustrates the payoff that can result.
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This page largely draws on the comprehensive “consensus guidance article” (236) for autonomic dysfunction by Dr. Svetlana Blitshteyn et al. This article is a goldmine of practical information. It guides clinicians through the diagnosis, testing, treatment, and lifestyle interventions for dysautonomia. Additionally, biases that impede access to care are discussed.
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The consensus guidance article was created to help clinicians manage post SARS-CoV-2 (Covid-19) related dysautonomia. The evaluation and management sections also apply to Sjogren’s dysautonomia.
Please print out the consensus guidance article (236) and refer to it as you read this page. Please contact me if you are have difficulty accessing the article.
REMINDER:
Please do not print out Sjogren’s Advocate pages and blog posts to use as clinician handouts. Learn why this can backfire by reading this blog post. The Clinician Handouts page shows you how to choose and share articles with your clinicians.
Do you have dysautonomia?
'Dysautonomia may cause or contribute to common Sjogren’s symptoms such as fatigue, brain fog, gastrointestinal dysmotility, and even sicca (dryness) (50). See Table 2 of the consensus guidance article (236, p.1273) for a more complete list of symptoms.
The Malmö POTS symptom score article (238) provides a self-assessment survey. You can take it to find out if you might have POTS. A score of 42 or higher indicates that POTS is likely. The score may also be high if you have any of the conditions causing orthostatic intolerance listed on Table 1 of the consensus guidance article (237, p.1272).
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Sjogren’s is a complex disease and symptoms often have multiple causes. For example, fatigue and exercise intolerance are common in Sjogren’s patients with or without dysautonomia. Dysautonomia is important to diagnose because it can be managed. A clinical evaluation should rule out dysautonomia mimics such as heart and lung disease.
The consensus guidance article focuses on POTS, orthostatic hypotension (OH), and other conditions that cause orthostatic intolerance.
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See the POTS page to learn how to do a standing test at home and for a variety of self-advocacy tools.
What about other types of dysautonomia symptoms?
Orthostatic intolerance is a broad term used to describe a condition that causes symptoms to worsen with upright posture.
Some symptoms, such as bladder problems, or gastrointestinal disorders cannot be explained by the inability to tolerate upright posture. Management of these other symptoms is not addressed in the consensus guidance article.
Success with Self-Advocacy:
Prepare for your visit ahead of time.
1. Record your symptoms to share with your doctor.
Print out the consensus guidance article and highlight symptoms that you have on Table 2.
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Keep a dysautonomia symptom log. Document the impact that your symptoms have on your function: ability to work, sit, stand, walk, exercise, do chores, cook, etc.
Be specific with your description. For example, how long can you stand in a line? What activities can you no longer do? What makes symptoms better or worse? If you have tried any of the lifestyle measures (page 1281 of the consensus guidance article), what impact do they have?
2. Perform two or three standing tests at home.
See the POTS page for instructions. Record your standing results and share them with your clinician. Abnormal results should prompt further evaluation, including a repeat standing test in the clinic.
If your home standing tests do not meet the diagnostic criteria for any of the conditions listed on Table 1 of the consensus guidance article but you have typical POTS symptoms, you can still be treated for orthostatic intolerance. If that is the case, highlight this sentence (my italics) on page 1272 to share with your clinician:
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“Orthostatic intolerance (OI) is used when objective tests do not confirm a diagnosis of one of the common autonomic disorders in a clinical setting of autonomic symptoms that are precipitated by an upright position and relieved by recumbency.”
3. Anticipate and prepare for misinformation and dismissive treatment.
I recommend creating a fan file with the following sections so that you can easily access these articles by topic:
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Consensus guidance (share with all clinicians)
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Sjogren’s and dysautonomia (item 2)
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Psych/ FND (Item 3)
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Deconditioning (item 4)
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Neg SSA/SSB (item 5 articles)
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Misc. (item 6 articles that you choose)
Top misperceptions: How to counter them
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1. Your clinician does not think that autonomic disorders are real or important.
Offer the consensus guidance article and seek a referral to a clinician who is willing to evaluate these conditions. You may want to join Smart Patients or a local Dysautonomia International support group to find referrals.
Print the PDF of this article, The Patient Perspective: What Postural Orthostatic Tachycardia Syndrome Patients Want You To Know, and highlight sections that you want to discuss with your doctor, as explained on the Handouts For Clinicians page.
Print this quote and share it with clinicians (or with friends and family)"
"To imagine POTS for yourself: number one, fast for 24 hours; number two, donate a pint of your blood; number three, go home and turn your thermostat up to 100 degree, number four, stand motionless. Enjoy the symptoms."
~ Dr. Jeffrey Boris, pediatric cardiologist, Children's Hospital of Philadelphia
2. Your clinician thinks that autonomic disorders are not a part of Sjogren’s disease.
Print the Dysautonomia and Sjogren's blog post by the Sjogren's Foundation, 10-26-2023
Print citation 50 and the abstract of citation 33 and share with your clinician.
Offer the consensus guidance article. Be sure to tell your clinician that even though the article was written for long Covid patients, the evaluation and treatment in this article pertains to Sjogren’s dysautonomia. If you have had COVID-19, even a mild case, this should be a moot point because POTs and other autonomic disorders are well documented outcomes of SARS-CoV2 infection.
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To understand the details of Sjogren's and dysautonomia, you may wish to watch this 2023 presentation by Lauren Stiles, CEO of Dysautonomia International with Q and A by Dr. Sarah Schafer and Jennifer Samghabadi, NP. ​Sjogren's-The second most common cause of dysautonomia,
3. Your clinician tells you that your symptoms are caused by anxiety, depression, or functional neurological disorder (FND).
Clinicians often default to psychological explanations when they do not understand a condition.
Be prepared to counter these comments giving them copies of these two articles, with the following sections highlighted (my italics):
Highlight this entire paragraph on page 1274 of the consensus guidance article:
Similarly, some patient with PASC related autonomic dysfunction may present with symptoms of anxiety... diagnosed with POTS.
Highlight these sentences on page 5 of Raj et al (237).
Raj et al. found that adult POTS patients did not have increased current or lifetime prevalence of anxiety disorders. POTS patients, however, scored as moderately anxious compared to the background population using a non-discriminating questionnaire that measured both somatic and psychological symptoms (Beck Anxiety Inventory). In contrast, POTS patients did not have elevated anxiety on a questionnaire weighted to measure psychological anxiety symptoms (Anxiety Sensitivity Index). They concluded that the clinical appearance of anxiety in POTS was likely related to the illness rather than representing an anxiety disorder.
4. Your clinician tells you that your symptoms are caused by deconditioning (being out of shape).
Share this 1-page article by Dr. Blitshteyn, a neurologist and dysautonomia expert.
5. You are negative for SSA and SSB. Your clinician tells you that only people with SSA or SSB get systemic manifestations.
This is not only untrue, but Sjogren’s patients without SSA or SSB are more likely to have dysautonomia and small fiber neuropathy (178, 184).
Print out citation 178, the entire article. Highlight the third paragraph of the abstract. Point out that only 42.5% of Sjogren’s patients with small fiber neuropathy were positive for SS-A.
6. For more topics, see the blog post: Is it POTS? Debunking myths.
What about treatment?
Dysautonomia management usually requires both medication and lifestyle interventions. If you have any form of orthostatic intolerance, you can use the recommendations on pages 1280-1285 of the consensus guidance article to discuss medical and lifestyle management with your clinician.
Medications often require a trial-and-error approach. Beta blockers such as low dose propranolol are common first-line drugs but are not a good choice for patients with orthostatic hypotension or chronic low blood pressure. There are many other medication options. Pyridostigmine has a side effect that often benefits people with Sjogren’s: it increases saliva production and bowel motility.
Dysautonomia is common but underdiagnosed in Sjogren’s disease. Patients now have many tools they can use to advocate for proper diagnosis and treatment.
Updated 04-12-2024