Ehlers-Danlos Syndrome and POTS: What's the Connection?

Ehlers-Danlos syndrome (EDS) and POTS often occur together. EDS is a connective tissue disorder that can affect joints, skin, blood vessels, and other parts of the body. POTS is a disorder of the autonomic nervous system that can cause dizziness, a fast heartbeat, fatigue, and brain fog when someone stands up. The type of EDS most often linked to POTS is hypermobile Ehlers-Danlos syndrome, or hEDS, and that is the focus here. Most of the research and clinical discussion around this overlap centers on hEDS and related hypermobility conditions.

For many people, learning about this connection is what finally makes years of confusing symptoms start to make sense.

It might begin with knee or elbow pain, repeated sprains, and unusual flexibility. Then later come new problems: dizziness, a racing heart, or brain fog every time you stand up. On the surface, these issues can feel completely unrelated, like separate problems piling up for no clear reason. But in many cases, they are more closely connected than they first appear.

Understanding the connection between EDS and POTS will not ease symptoms overnight. It can, however, give people a better place to start.

What is Ehlers-Danlos syndrome?

Ehlers-Danlos syndrome is a group of inherited connective tissue disorders. Connective tissue is what holds together and supports structures throughout the body: joints, skin, blood vessels and organs. And a major component of connective tissue is collagen, which acts as a supportive framework.

There are 13 recognized types of EDS, but the one most often discussed alongside POTS is hypermobile EDS (hEDS). People with hEDS tend to have joints that move well beyond normal range, along with chronic pain, joint instability, frequent sprains, subluxations or dislocations, and fatigue. Some also have unusually soft or velvety skin. Unlike most other EDS types, hEDS does not yet have a confirmed genetic test. Diagnosis relies on clinical criteria rather than lab results.

Not everyone who is flexible has EDS, though. Some people have naturally loose joints with no related illness. Others fall into a category called hypermobility spectrum disorder (HSD), where hypermobility causes real symptoms but the person does not meet the full criteria for hEDS. That distinction matters here because people with HSD can experience POTS-like symptoms too.

What is POTS?

POTS stands for postural orthostatic tachycardia syndrome. It is a disorder of the autonomic nervous system — the part of the body that automatically manages heart rate, blood pressure, digestion, and temperature regulation.

In plain terms: when a person with POTS stands up, the body does not adjust blood flow the way it should. Blood pools in the lower body instead of returning efficiently to the heart and brain. To compensate, the heart speeds up. In adults, POTS is generally defined by a heart rate increase of 30 beats per minute or more within 10 minutes of standing, without a significant drop in blood pressure.

But POTS involves much more than a fast heartbeat. Common symptoms include:

• dizziness or lightheadedness
• palpitations
• fainting or near-fainting
• fatigue
• brain fog
• nausea
• headaches
• exercise intolerance
• chest discomfort
• blood pooling in the legs

One thing that frustrates many patients is how often POTS gets mistaken for anxiety. The confusion is understandable — a racing heart, dizziness, and fatigue are symptoms that anxiety and POTS share. But POTS is not anxiety. Unlike anxiety, POTS comes with measurable heart rate changes and documented autonomic dysfunction that show up in testing. Most patients have structurally normal hearts, and research has found that the clinical appearance of anxiety in POTS is largely driven by somatic symptoms that overlap with common anxiety symptoms, not by a higher rate of anxiety disorders. One study found that POTS patients did not have an increased current or lifetime prevalence of anxiety disorders compared to the general population. Despite that, many spend years being told their symptoms are psychological before ever getting a correct diagnosis.

Why do EDS and POTS often show up together?

The connection between hEDS and POTS is well recognized at this point, though researchers describe it as a strong association rather than a fully proven cause-and-effect relationship.

The numbers are hard to ignore. In one study evaluating 91 POTS participants using the 2017 hEDS diagnostic criteria, 31% met the clinical criteria for hEDS, and an additional 24% had generalized joint hypermobility without meeting the full hEDS threshold. The exact figures shift depending on the study, but the pattern is consistent: these two conditions show up together a lot.

Why? Researchers have a few working theories.

Blood vessels that stretch too much. In hEDS, connective tissue is looser than it should be throughout the whole body — and that includes the walls of blood vessels. If the veins in your legs and abdomen are too stretchy, they have trouble pushing blood back up toward your heart when you stand. Blood pools in the lower body instead, and your heart speeds up trying to compensate. This is probably the most straightforward explanation for why the two conditions overlap.

Small fiber neuropathy. Some people with hEDS also have damage to the tiny nerve fibers responsible for things like pain, temperature, and keeping blood vessels properly tightened. One study of 79 hEDS/HSD patients found that 78% had reduced intraepidermal nerve fiber density, a structural marker for small fiber neuropathy. If those nerves are not doing their job, the body struggles to make the quick adjustments that standing up demands.

Slow autonomic reflexes. When blood vessels are overly compliant, the pressure sensors that normally catch a drop in blood flow may respond too slowly. The adjustments that should happen within seconds of standing get delayed, and symptoms follow.

Mast cell involvement. Some patients also deal with mast cell activation syndrome, or MCAS, and some clinicians informally refer to hEDS, POTS, and MCAS together as a triad. Mast cells release chemicals like histamine that can widen blood vessels and worsen things like flushing, digestive problems, and blood pooling. One study found that 31% of patients with both POTS and EDS also had MCAS, compared to just 2% of patients without POTS and EDS. However, the evidence base for this triad is still developing — a 2020 literature review noted that a common pathophysiologic mechanism connecting all three conditions has yet to be established, and the diagnostic criteria for MCAS remain debated.

These explanations are not mutually exclusive. For many people, more than one of these mechanisms is probably contributing at the same time.

What it feels like when both conditions are present

When hEDS and POTS overlap, the combined effect can be more disruptive than either condition alone. The symptoms tend to feed into each other.

A person already dealing with joint instability, chronic pain, and fatigue from hEDS now also has dizziness, a racing heart, brain fog, and trouble tolerating standing. Everyday things — showering, cooking, standing in line at a store, walking through a hot parking lot — can become exhausting in ways that are hard to explain to people who have not experienced it.

Where the two conditions compound each other most:

• fatigue that goes beyond what either condition alone would cause
• exercise intolerance made worse by joint instability
• brain fog layered on top of chronic pain
• temperature sensitivity triggering both POTS flares and joint inflammation
• sleep problems from pain, palpitations, or both
• nausea and digestive issues from autonomic dysfunction and connective tissue laxity in the gut

Mornings tend to be tough. Heat, dehydration, large meals, prolonged standing, and overexertion can all trigger flares. And because both conditions are often invisible to others, people generally are unaware of what someone with hEDS and POTS is dealing with day to day.

How are EDS and POTS diagnosed?

POTS is typically diagnosed through a combination of symptoms and objective testing. The most well-known test is the tilt table test, where the patient lies on a table that slowly tilts from flat to upright while heart rate and blood pressure are monitored throughout. Some clinicians use an active standing test instead, which measures the same changes but has the patient simply move from lying down to standing on their own.

hEDS is diagnosed clinically, meaning there is no lab test or scan that confirms it — doctors rely on the 2017 diagnostic criteria, which look at generalized joint hypermobility, other connective tissue features, and the ruling out of similar conditions. You may come across the Beighton score, which is a simple way of measuring hypermobility, but it is just one piece of a larger picture. There is currently no genetic test for hEDS.

Because both conditions can affect so many different parts of the body, getting a diagnosis often means seeing multiple specialists. Depending on what is going on, that could include a cardiologist, autonomic neurologist, geneticist, rheumatologist, physical therapist, pain specialist, or gastroenterologist.

For many patients, the most frustrating part is simply how long the whole process takes. Spending years bouncing between doctors before anyone connects the dots is, unfortunately, not unusual.

Treatment and management

There is no cure for hEDS or POTS. Treatment is about managing symptoms and making daily life more functional. Most plans start with conservative, non-medication approaches.

For POTS specifically, common first steps include:

• drinking significantly more fluids
• increasing salt intake (under medical guidance)
• wearing compression garments
• avoiding known triggers like heat and dehydration
• eating smaller, more frequent meals
• starting a gradual, structured exercise program

Exercise is one of the most effective tools for managing POTS — but when hEDS is also in the picture, it has to be approached carefully. You cannot just jump on a treadmill and push through it.

Most structured programs, like the Levine Protocol (also called the Dallas Protocol) or the CHOP Modified Protocol developed at the Children's Hospital of Philadelphia, start with recumbent exercise: rowing machines, recumbent bikes, swimming, or water aerobics. The idea is to build cardiovascular fitness while keeping the body horizontal, so the heart is not fighting gravity from the start. Over time — typically over several months — more upright activity gets added gradually. Rushing that progression tends to backfire.

For someone with hEDS, there is an extra layer to think about — joint stability. High-impact activities like running or jumping, aggressive stretching, and any movements that push joints past their natural range can cause more harm than good. The goal with physical therapy is not to increase flexibility, which most hEDS patients already have too much of, but to build the muscle support around unstable joints. Controlled strengthening exercises, done with good form and proper guidance, tend to work far better than generic workout plans.

When lifestyle changes are not enough on their own, medications can help fill the gap. Some target heart rate, slowing it down so the body is not constantly racing to compensate. Others help blood vessels constrict more effectively, or work to increase blood volume so there is more to work with in the first place — extra salt and fluid intake, or medications like fludrocortisone, are common examples of this approach. What works varies considerably from person to person, and finding the right combination often takes time.

Practical tips for daily life

Living with both hEDS and POTS usually means making a lot of small adjustments. None of them are dramatic on their own, but they add up:

• drink water before getting out of bed in the morning
• stand up slowly, especially after lying down
• use a shower chair and cooler water temperatures
• plan demanding tasks for the time of day when you feel best
• wear compression stockings or abdominal compression
• track triggers and patterns in a symptom journal
• pace yourself to avoid crash cycles
• keep water, electrolytes, snacks, and any medications within reach when you are out

Some people also find it helpful to learn counter-maneuvers for standing — crossing the legs, tightening leg muscles, or shifting weight from one foot to the other. Small things, but they can make a real difference when you are stuck standing in a line or waiting somewhere.

Common myths worth clearing up

"POTS is just anxiety." No. Anxiety can coexist with chronic illness, but it does not cause the measurable autonomic dysfunction that defines POTS. Research has shown that the symptoms in POTS are phenomenologically distinct from panic disorder, and that POTS patients do not have anxiety disorders at unusually high rates. This misconception delays diagnosis for a lot of people.

"If you're flexible, you have EDS." Not necessarily. Plenty of people have naturally loose joints with no illness. Others have HSD, which is different from hEDS. Flexibility alone is not a diagnosis.

"If you look fine, you are fine." Both hEDS and POTS are often invisible. A person can appear healthy while dealing with serious pain, dizziness, fatigue, and cognitive problems.

Frequently asked questions

Can EDS cause POTS? EDS does not directly cause POTS, but the two are strongly associated, especially in people with hypermobile EDS. The connective tissue abnormalities in hEDS — like overly stretchy blood vessels — are thought to contribute to the autonomic dysfunction seen in POTS.

What percentage of EDS patients have POTS? Estimates vary, but research suggests that roughly two-thirds of people with hEDS experience some form of orthostatic intolerance, with 41–49% meeting the criteria for POTS specifically.

Is POTS curable? There is no cure for POTS, but symptoms can often be managed effectively with lifestyle changes, exercise programs, and sometimes medication. Some people, especially those whose POTS was triggered by a viral illness, do see improvement over time.

What is the hEDS/POTS/MCAS triad? Some patients with hEDS and POTS also develop mast cell activation syndrome (MCAS). Clinicians sometimes refer to this as a triad because the three conditions appear together more often than expected. However, the evidence for a shared underlying mechanism is still limited, and the diagnostic criteria for MCAS are still being refined.

What kind of doctor should I see? Diagnosis and management often involve multiple specialists. A cardiologist or neurologist can evaluate POTS, while a geneticist or rheumatologist typically handles the EDS side. A knowledgeable physical therapist is often essential for both. Our directory can help you find providers who understand these conditions.

When to consider medical evaluation

It is worth talking to a doctor if you experience repeated dizziness when standing, an unexplained fast heartbeat when upright, fainting episodes, unusual joint flexibility along with pain or instability, or a combination of symptoms that suggests both hypermobility and problems with standing tolerance.

Bringing a written symptom history, family history, and notes about what makes symptoms better or worse can help a lot. Objective testing matters — especially for patients who have been dismissed with explanations like "it's just stress."

The bottom line

The connection between Ehlers-Danlos syndrome and POTS is real, and it is especially common in people with hypermobile EDS and related hypermobility conditions. The exact mechanisms are still being studied, but the overlap is well established and taken seriously by the medical community.

For a lot of people, discovering this connection is the moment things start to click. Symptoms that seemed random start to fit a pattern. And that understanding — even before treatment changes — can be a relief.

If you are looking for a specialist who understands both EDS and POTS, our directory lists providers across the country who work with these conditions. You can search by location, specialty, or condition treated.

Sources

• Miller AJ, Stiles LE, Sheehan T, et al. "Prevalence of Hypermobile Ehlers-Danlos Syndrome in Postural Orthostatic Tachycardia Syndrome." Autonomic Neuroscience, 2020; 224:102637.
• Malfait F, et al. "The 2017 International Classification of the Ehlers-Danlos Syndromes." American Journal of Medical Genetics Part C, 2017.
• Vernino S, et al. "Postural Orthostatic Tachycardia Syndrome (POTS): State of the Science and Clinical Care from a 2019 National Institutes of Health Expert Consensus Meeting — Part 1." Autonomic Neuroscience, 2021; 235:102828.
• Raj SR, et al. "Diagnosis and Management of Postural Orthostatic Tachycardia Syndrome." CMAJ, 2022; 194(10):E378–E385.
• Sheldon RS, et al. "2015 Heart Rhythm Society Expert Consensus Statement on the Diagnosis and Treatment of Postural Tachycardia Syndrome, Inappropriate Sinus Tachycardia, and Vasovagal Syncope." Heart Rhythm, 2015; 12(6):e41–e63.
• Fernandez A, et al. "Small Fiber Neuropathy in Hypermobile Ehlers-Danlos Syndrome / Hypermobility Spectrum Disorder." Journal of Internal Medicine, 2022; 292(3):522–524.
• Fu Q, Levine BD. "Exercise and Non-Pharmacological Treatment of POTS." Autonomic Neuroscience, 2018; 215:20–27.
• Kohn A, Chang C. "The Relationship Between Hypermobile Ehlers-Danlos Syndrome (hEDS), Postural Orthostatic Tachycardia Syndrome (POTS), and Mast Cell Activation Syndrome (MCAS)." Clinical Reviews in Allergy & Immunology, 2020; 58(3):273–297.
• Dempsey A, et al. "The Relationship Between Mast Cell Activation Syndrome, Postural Tachycardia Syndrome, and Ehlers-Danlos Syndrome." Allergy and Asthma Proceedings, 2021; 42(3):243–246.
• Raj V, et al. "Cognitive and Psychological Issues in Postural Tachycardia Syndrome." Autonomic Neuroscience, 2018; 215:46–55.
• Masuki S, et al. "Excessive Heart Rate Response to Orthostatic Stress in Postural Tachycardia Syndrome Is Not Caused by Anxiety." Journal of Applied Physiology, 2007; 102:1134–1142.
• The Ehlers-Danlos Society. Hypermobile EDS and Dysautonomia.
• Dysautonomia International. Postural Orthostatic Tachycardia Syndrome.