Could you have POTS?
(Postural Orthostatic Tachycardia Syndrome)

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You might have POTS if you have many of these symptoms

*Lightheadedness with upright posture
 

*Standing intolerance
 

*Pounding heart, palpitations, chest discomfort

Severe fatigue
 

Brain fog
 

Tremulousness
 

Nausea and other GI symptoms
 

Severe frequent headaches/ migraines
 

These are some of the most common POTS symptoms. If you have many of these symptoms, especially the first three (*), for more than a few months, you may have POTS or other autonomic disorders (dysautonomia).

What is POTS?

POTS is one of the most common neurologic complications of Sjogren's. Like Sjogren’s, POTS impacts about 1% of the population, mostly women.


The following explanation of POTs is oversimplified. However, it provides is a useful model for understanding POTS diagnosis and management.

A simple explanation of POTS

One job of the autonomic nervous system (ANS) is to regulate blood flow in the body. The body normally adapts to upright posture because the ANS signals blood vessels in the legs and abdomen to constrict in response to standing. This allows blood to return to the heart from the lower body so it can be pumped out again.

In POTS, this squeezing action on the blood vessels does not work well. With standing, blood pools in the lower body. This reduces blood available to flow to the lungs, upper body, and brain. In an attempt to compensate, the heart rate keeps going up. Catecholamines (stress hormones) may be released, causing people with POTS to feel shaky. Symptoms such as lightheadedness, brain fog, and a pounding heart sensation worsen over time with standing.  

POTS is a common complication of Sjogren's

Knowing about POTS-Sjogren’s connection is important because a lot can be done to manage POTS and improve quality of life. The exact number of people who have both Sjogren’s and POTS is unknown because most people with these conditions remain undiagnosed.
 

Sjogren’s diagnosis often comes very late in people with POTS and other neurologic Sjogren’s features. This group is often negative for SS-A and other antibodies associated with Sjogren’s. Neurologic features usually precede sicca symptoms/dryness (161). Many clinicians will not diagnose Sjogren’s in antibody-negative patients, even though seronegative Sjogren’s is common. Even fewer clinicians will consider a Sjogren’s diagnosis without obvious sicca. This is unfortunate because a POTS patient with Sjogren’s is likely to benefit from Sjogren’s-specific treatments (33).

Nerve conduction studies

These tests evaluate the electrical conduction of the myelinated motor and sensory nerves. Because they do not test for small unmyelinated nerve function, they cannot rule out SFN, SFPN, or dysautonomia.  

We should know more about the relationship between Sjogren’s and POTS in a few years, thanks to a new study being funded through a collaborative effort of the Sjogren’s Foundation and Dysautonomia International.

Missed and dismissed

Sjogren’s disease and POTS are physiologic conditions, not emotional disorders. Yet both tend to be misdiagnosed as anxiety (or worse, conversion disorder) before a proper diagnosis is finally made. Anxiety does not cause Sjogren’s or POTS. Obviously, not being able to find help for a serious disease can be anxiety-provoking.  

"PoTS can be misdiagnosed for a number of reasons. Patients may repeatedly present with a multitude of symptoms, often without obvious clinical findings: typical ‘heart-sink’ patients. Although severely incapacitated, they often appear well. Patients may not recognise the significance of symptoms or be reluctant to divulge them for fear of the clinician's response. In consultations, blood pressure and heart rate measurements are usually taken with patients in the seated position, when recordings may be normal.

Experienced physicians and patient support groups are aware that patients often acquire inappropriate psychiatric diagnoses and consequently, distrust of doctors is common. Interestingly, using an anxiety assessment tool that relies on cognitive symptoms as opposed to somatic ones, PoTS patients have a tendency to lower anxiety levels than controls (190)."
 

How do you get diagnosed with POTS?

Diagnosis can be challenging because many clinicians remain unfamiliar with POTS. Some people are diagnosed by a neurologist, cardiologist, or primary care provider (PCP). Sometimes the best strategy is finding a PCP who is willing to learn about POTS.
 

Before you seek diagnosis, it may be helpful to screen yourself several times at home using the instruction on this PDF. 

 

 

POTS Screening:

Standing Test Instructions

How to do a standing test at home for POTS
An abbreviated version of the PDF instructions

  • Use a reliable electronic heart rate/ blood pressure monitor.
     

  • Set everything up in advance. Create a chart for recording heart rate and blood pressure: supine, 1 minute, 5 minutes, 10 minutes.
     

  • Document the date and time of day that each test is done.
     

  • Rest at least 5 minutes supine (lying on your back) before taking your initial resting heart rate and blood pressure.
     

  • Stand completely still during the 10 minutes. It is best to have someone else write record for you; every movement decreases test accuracy.
     

  • Do not wear tight clothing or compression garments during the test.

    Stop the test right away and lie down if you feel like fainting.

Share these four items with your clinician:

1. your home standing test results, documented from 2 or 3 sessions

2. the PDF standing test instructions

3. this POTS review article (190)
4. a printout of this page from the POTS UK website

The standing test should be repeated by a health care professional in the clinic. They may find the instruction handout helpful.

The current criteria for POTS diagnosis

Adults 20 and older: A sustained increase in heart rate of 30 beats per minute (bpm) or higher within 10 minutes of standing is considered positive for POTS.

Teens 12-19: a sustained increase in heart rate elevation of 40 bpm or higher within 10 minutes of standing.

 

A note about blood pressure:

The blood pressure in POTS is usually stable, although this varies. It is important to record both heart rate and blood pressure because orthostatic hypotension may also cause a rapid heart rate with standing.

The standing test is not perfect.

  • It can be tricky to stand completely still, especially if you are recording your own results.
     

  • Results may vary with hydration, salt intake, time of day, air temperature, recent exercise, and other factors.
     

  • A tilt table test is helpful when there is a sustained heart rate increase (such as 20-29 bpm) that does not make the 30 bpm cutoff. The tilt table removes the inaccuracies caused by spontaneous leg muscle contractions during standing. Very few medical centers have a tilt table available.
     

  • The 30 bpm cutoff is arbitrary, but generally agreed upon. Some people have a clear POTS-like clinical picture but do not meet the 30 bpm cutoff. Researchers are looking for more accurate tests because this condition is not simply a heart rate problem.  
     

The information on this page, or any page of Sjogren’s Advocate, does not substitute for a formal diagnosis or medical advice. If you think you have POTS or another autonomic disorder, it is important to discuss this with your clinician.  Please read the disclaimers page if you have not already done so.
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RESOURCES

Most of these links are listed on the Resources page for Dysautonomia/ POTS.

ARTICLES FOR CLINICIANS

Good overview of POTS (190)
Excellent description of POTS and POTS diagnosis.  Open Access.
 

Medications used for POTS(191)

A thorough review of treatments, including doses and strategies. This article is not open access. If you don’t wish to purchase it, you may request it from a medical library or copy the abstract for your clinician.

ARTICLES FOR  PATIENTS

Managing POTS symptoms. 
Scroll to the end of this article for a brief summary of lifestyle measures. There are a few minor inaccuracies in the article, overall, a good read.  
An important addition to lifestyle measures: avoid heat exposure, especially hot tubs, saunas, hot baths, long showers, and warm pools. These cause vasodilation and blood pooling.
 

How to exercise safely with POTS and/or severe Sjogren’s fatigue

Managing fatigue

A good description of pacing which also benefits Sjogren’s fatigue without POTS. 

WEBSITES

Dysautonomia international – annual patient conferences, no cost 
 

Sjogren’s Advocate- The Dysautonomia main page  and the Dysautonomia/ POTS resources page
 

The Dysautonomia Project - new website, many sections remain incomplete. The book by the same name (Freeman, Goldstein, Thompson et al) is packed with information for both clinicians and patients.

Updated 10-12-2021