Ehlers-Danlos Syndrome and POTS: The Connection

Learn about Ehlers-Danlos Syndrome (EDS) and POTS, their shared symptoms, diagnosis, and treatment options. Discover how they are connected.

March 14, 2025

Ehlers-Danlos Syndrome (EDS) and Postural Orthostatic Tachycardia Syndrome (POTS) are two chronic, often overlapping conditions that can significantly impact a person’s quality of life. While they are distinct disorders, their frequent coexistence has led researchers to explore the intricate relationship between them. This article delves into the nature of EDS and POTS, their symptoms, the link between them, and how they are diagnosed, treated, and managed.

What is Ehlers-Danlos Syndrome (EDS)?

Ehlers-Danlos Syndrome is a group of inherited connective tissue disorders caused by defects in collagen, a protein that provides structure and strength to skin, joints, blood vessels, and other tissues. Collagen acts as the “glue” that holds the body together, and when it is compromised, it leads to a wide range of symptoms. There are 13 recognized subtypes of EDS, with hypermobile EDS (hEDS) being the most common. Other subtypes include classical EDS, vascular EDS, and kyphoscoliotic EDS, each with its own unique features and genetic causes.

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Common Symptoms of EDS:

Joint Hypermobility: Joints that move beyond the normal range of motion, leading to frequent dislocations or subluxations.
Skin Hyperextensibility: Skin that is soft, stretchy, and fragile, often bruising easily or tearing with minor trauma.
Chronic Pain: Persistent joint and muscle pain due to instability and overuse of joints.
Tissue Fragility: Poor wound healing and abnormal scarring, such as atrophic or “cigarette paper” scars.
Fatigue: Chronic fatigue, often exacerbated by pain and sleep disturbances.
Gastrointestinal Issues: Conditions like gastroparesis (delayed stomach emptying), irritable bowel syndrome (IBS), and chronic constipation or diarrhea.
Cardiovascular Complications: Mitral valve prolapse, aortic root dilation, and other structural heart issues.

EDS is often underdiagnosed or misdiagnosed due to its wide range of symptoms and the lack of a definitive genetic test for the hypermobile subtype (hEDS). Diagnosis typically relies on clinical evaluation, family history, and assessment of specific diagnostic criteria.

What is Postural Orthostatic Tachycardia Syndrome (POTS)?

Postural Orthostatic Tachycardia Syndrome (POTS) is a form of dysautonomia, a disorder of the autonomic nervous system. The autonomic nervous system regulates involuntary bodily functions such as heart rate, blood pressure, digestion, and temperature control. POTS is characterized by an abnormal increase in heart rate upon standing, often accompanied by a drop in blood pressure or blood pooling in the lower extremities.

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Common Symptoms of POTS:

Rapid Heart Rate (Tachycardia): A heart rate increase of 30 beats per minute (or 40 beats per minute in adolescents) within 10 minutes of standing.
Dizziness and Lightheadedness: Feeling faint or unsteady when standing, often relieved by sitting or lying down.
Fainting (Syncope): Loss of consciousness due to a sudden drop in blood pressure or inadequate blood flow to the brain.
Chronic Fatigue: Overwhelming tiredness that is not relieved by rest.
Brain Fog: Difficulty concentrating, memory issues, and mental cloudiness.
Gastrointestinal Symptoms: Nausea, bloating, abdominal pain, and other Ehlers-Danlos and GI symptoms.
Exercise Intolerance: Difficulty engaging in physical activity due to symptoms like rapid heart rate, dizziness, or fatigue.
Cold or Discolored Extremities: Poor circulation leading to cold hands and feet or a bluish tint to the skin.

POTS can be triggered by various factors, including viral infections, surgery, trauma, or hormonal changes. It is often misdiagnosed as anxiety or chronic fatigue syndrome due to its wide range of symptoms.

The Link Between EDS and POTS

The connection between EDS and POTS is well-documented, with studies showing that up to 80% of individuals with hypermobile EDS (hEDS) also experience POTS. Conversely, about 31% of people with POTS have hEDS. This significant overlap suggests shared underlying mechanisms, though the exact relationship is still being studied.

Possible Explanations for the Link:

Connective Tissue Dysfunction: In EDS, weakened connective tissue may affect the integrity of blood vessels, leading to poor blood flow regulation. This can contribute to the blood pooling and orthostatic intolerance seen in POTS.
Autonomic Nervous System Dysfunction: Both conditions involve dysregulation of the autonomic nervous system. In EDS, structural abnormalities in connective tissue may disrupt nerve and blood vessel function, exacerbating POTS symptoms.
Hypermobility and POTS: POTS and hypermobility often coexist, as the instability of joints and tissues in EDS may further strain the autonomic nervous system.
Shared Genetic Factors: Some researchers believe that EDS and POTS may share underlying genetic predispositions, though this area requires further study.
Secondary Complications: Chronic pain, fatigue, and deconditioning associated with EDS can worsen POTS symptoms, creating a cycle of symptom exacerbation.

Diagnosing EDS and POTS

Diagnosing EDS and POTS can be challenging due to their overlapping symptoms and the lack of definitive diagnostic tests for hEDS. A thorough clinical evaluation is essential.

For EDS, diagnosis often involves:

A detailed medical history and physical examination
Assessment of joint hypermobility using tools like the Beighton Score
Evaluation of skin texture, tissue fragility, and other connective tissue manifestations
Genetic testing for certain subtypes (though not available for hEDS)

For POTS, diagnosis typically includes:

Heart rate monitoring during positional changes (e.g., lying down to standing)
Blood tests to rule out other conditions, such as anemia or thyroid disorders
A tilt table test, which measures heart rate and blood pressure changes while the patient is tilted upright

Treatment and Management

While there is no cure for EDS or POTS, treatment focuses on managing symptoms and improving quality of life. A multidisciplinary approach is often necessary.

For EDS, management may include:

Physical Therapy: To stabilize joints, improve strength, and prevent dislocations.
Pain Management: Medications, acupuncture, or other therapies to address chronic pain.
Surgical Interventions: For severe joint instability or other complications.
Lifestyle Modifications: Avoiding high-impact activities that could strain joints.

For POTS, treatment often involves:

Increased Fluid and Salt Intake: To improve blood volume and reduce symptoms.
Compression Garments: To prevent blood pooling in the lower extremities.
Medications: Beta-blockers, ivabradine, or midodrine to regulate heart rate and blood pressure.
Gradual Exercise Programs: To improve cardiovascular fitness and reduce deconditioning.

Both conditions benefit from a personalized treatment plan tailored to the individual’s specific needs. Psychological support, such as counseling or therapy, can also help address the emotional challenges of living with chronic illness.

Living with EDS and POTS

Living with EDS and POTS can be physically and emotionally challenging. The unpredictable nature of symptoms and the need for ongoing management can make daily life difficult. However, with proper diagnosis, treatment, and support, many individuals are able to manage their symptoms and lead fulfilling lives.

Key Tips for Living with EDS and POTS:

Pace Yourself: Avoid overexertion and listen to your body.
Stay Hydrated: Drink plenty of fluids and increase salt intake if recommended by your doctor.
Use Mobility Aids: Canes, braces, or wheelchairs can help reduce strain on joints.
Seek Support: Connect with healthcare professionals, support groups, or online communities.
Focus on Mental Health: Counseling or therapy can help address the emotional impact of chronic illness.

Conclusion: Ehlers-Danlos Syndrome and POTS

Ehlers-Danlos Syndrome and Postural Orthostatic Tachycardia Syndrome are distinct yet often interconnected conditions. EDS affects connective tissue, leading to joint hypermobility and tissue fragility, while POTS disrupts the autonomic nervous system, causing dizziness and rapid heart rate upon standing. Their frequent coexistence underscores the importance of a comprehensive, multidisciplinary approach to diagnosis and treatment.

By raising awareness and advancing research, we can improve the lives of those living with EDS and POTS, offering hope for better management and quality of life. If you suspect you have EDS, POTS, or both, consult a healthcare professional for a thorough evaluation and personalized care plan.

FAQs About Ehlers-Danlos Syndrome and POTS

What is the EDS-POTS-MCAS Triad?

The EDS-POTS-MCAS triad refers to the common coexistence of Ehlers-Danlos Syndrome, Postural Orthostatic Tachycardia Syndrome, and Mast Cell Activation Syndrome (MCAS). MCAS involves abnormal mast cell activity, leading to symptoms like allergic reactions, gastrointestinal issues, and skin rashes.

Does EDS or POTS Affect Life Expectancy?

Most forms of EDS, including hEDS, do not significantly affect life expectancy. Similarly, POTS is not life-threatening on its own. However, complications from either condition, such as cardiovascular issues, may require careful management.

Can You Exercise with EDS and POTS?

Yes, but exercise should be approached cautiously. Low-impact activities like swimming, yoga, or using a recumbent bike are often recommended. Starting slowly and working with a physical therapist can help build tolerance.