The link betwen EDS and POTS

Ehlers-Danlos Syndrome (EDS) and Postural Orthostatic Tachycardia Syndrome (POTS) often coexist, presenting a complex clinical picture that can significantly impact patients' quality of life. Understanding the overlap between these conditions is crucial for effective diagnosis and management.

Understanding Ehlers-Danlos Syndrome (EDS) and Postural Orthostatic Tachycardia Syndrome (POTS)

Ehlers-Danlos Syndrome (EDS) is a group of connective tissue disorders characterized by joint hypermobility, skin hyperextensibility, and tissue fragility. The most common type, hypermobile EDS (hEDS), involves symptoms like chronic pain, fatigue, and gastrointestinal issues.

Postural Orthostatic Tachycardia Syndrome (POTS) is a form of dysautonomia that affects blood flow, causing symptoms like dizziness, fainting, and rapid heart rate when moving from a lying down to a standing position. This condition is particularly prevalent in adolescents and young adults.

The Connection Between EDS and POTS

Patients with EDS, particularly hEDS, frequently report symptoms of POTS. Research indicates a significant overlap between these conditions, with a high percentage of EDS patients also meeting the criteria for POTS. This co-occurrence can complicate the clinical presentation and make management more challenging.

Mechanisms Behind the Overlap:

1. Connective Tissue Abnormalities: EDS affects the connective tissues that support the autonomic nervous system, potentially leading to the dysregulation seen in POTS.
2. Blood Vessel Compliance: Increased laxity in the blood vessels in EDS patients can result in abnormal blood pooling in the lower extremities, contributing to the orthostatic intolerance observed in POTS.
3. Joint Hypermobility: The physical stress from joint instability in EDS might trigger or exacerbate the autonomic dysfunction characteristic of POTS.

Symptoms and Diagnosis

Patients with both EDS and POTS often experience a range of symptoms, including:

• Orthostatic Intolerance: Difficulty standing for prolonged periods, dizziness, and fainting.
• Gastrointestinal Issues: Bloating, nausea, and altered bowel habits.
• Chronic Pain and Fatigue: Widespread pain and persistent fatigue impacting daily activities.
• Cognitive Dysfunction: "Brain fog," memory issues, and concentration difficulties.

Diagnosing these overlapping conditions requires a comprehensive approach. The Beighton scoring system is used to assess joint hypermobility for EDS, while a tilt table test or a 10-minute stand test can help diagnose POTS.

Management Strategies

Effective management of patients with both EDS and POTS requires a multidisciplinary approach:

1. Medications:
   • Beta-Blockers: Help manage heart rate.
   • Fludrocortisone: Increases blood volume to reduce orthostatic symptoms.
   • Midodrine: Constricts blood vessels to improve blood flow.
2. Lifestyle Modifications:
   • Hydration and Diet: High salt and fluid intake to increase blood volume.
   • Compression Garments: Support blood flow in the lower extremities.
   • Physical Therapy: Tailored exercises to improve joint stability and cardiovascular conditioning.
3. Psychological Support:
   • Cognitive Behavioral Therapy (CBT): Addresses the psychological impact of chronic illness.
   • Support Groups: Provide community and shared experiences.

The Role of Multidisciplinary Care

Given the complexity of managing EDS and POTS, patients benefit from a team approach involving:

• Rheumatologists: For joint and connective tissue management.
• Cardiologists: For POTS and other cardiovascular issues.
• Gastroenterologists: To address gastrointestinal symptoms.
• Neurologists: For autonomic dysfunction and neurological symptoms.
• Psychologists or Psychiatrists: For mental health support.

Conclusion

Understanding and managing the overlap between EDS and POTS is crucial for improving patient outcomes. A comprehensive, multidisciplinary approach can help address the wide range of symptoms and improve the quality of life for those affected by these complex conditions.

‍

Frequently Asked Questions

What is the connection between EDS, POTS, and MCAS?

EDS, Postural Orthostatic Tachycardia Syndrome (POTS), and Mast Cell Activation Syndrome (MCAS) often coexist. POTS involves issues with blood flow and heart rate when moving from lying down to standing, while MCAS results in allergic-like reactions without a known allergen. These conditions together can complicate diagnosis and treatment, requiring a comprehensive approach.

What autoimmune disease is associated with Ehlers-Danlos syndrome?

There is no direct association between EDS and autoimmune diseases, but some patients report coexisting autoimmune conditions. The relationship is not fully understood and requires further research.

What is the trifecta Ehlers-Danlos syndrome?

The trifecta refers to the co-occurrence of EDS, POTS, and MCAS, which presents a complex clinical picture due to the overlap of symptoms affecting multiple systems.

What is the life expectancy of someone with EDS and POTS?

Life expectancy can vary depending on the EDS subtype and the presence of complications. For most people with hEDS, life expectancy is normal, but regular monitoring and management of symptoms are crucial.

Can you have both POTS and EDS?

Yes, many individuals with EDS also have POTS. Symptoms of POTS can include dizziness, fainting, and rapid heart rate upon standing, which can overlap with symptoms of EDS.

What is the EDS POTS triad?

The EDS POTS triad refers to the combination of EDS, POTS, and MCAS, which can significantly impact a patient's quality of life and require a coordinated treatment approach.

‍

References

1. Miglis, M. G., Schultz, B., & Muppidi, S. (2017). Postural tachycardia in hypermobile Ehlers-Danlos syndrome: A distinct subtype? Autonomic Neuroscience, 224, 102637. https://doi.org/10.1016/j.autneu.2020.102637
2. Grigoriou, E., Boris, J. R., & Dormans, J. P. (2015). Postural Orthostatic Tachycardia Syndrome (POTS): Association with Ehlers-Danlos Syndrome and Orthopaedic Considerations. Clinical Orthopaedics and Related Research, 473(2), 722-728. https://doi.org/10.1007/s11999-014-3898-x
3. Miller, A. J., Stiles, L. E., Sheehan, T., Bascom, R., Levy, H. P., Francomano, C., & Arnold, A. C. (2020). Prevalence of Hypermobile Ehlers-Danlos Syndrome in Postural Orthostatic Tachycardia Syndrome. Autonomic Neuroscience, 224, 102637. https://doi.org/10.1016/j.autneu.2020.102637
4. Tinkle, B., Castori, M., Berglund, B., Cohen, H., Grahame, R., Kazkaz, H., & Levy, H. (2017). Hypermobile Ehlers-Danlos syndrome (a.k.a. Ehlers-Danlos syndrome Type III and Ehlers-Danlos syndrome hypermobility type): Clinical description and natural history. American Journal of Medical Genetics Part C: Seminars in Medical Genetics, 175(1), 48-69. https://doi.org/10.1002/ajmg.c.31538

‍