People with hypermobile Ehlers-Danlos syndrome struggle to perceive their lung volume accurately, explaining why many experience unexplained breathing difficulties despite normal pulmonary function tests.
At a Glance
- 85% of adults with hypermobile Ehlers-Danlos syndrome (hEDS) report functional respiratory complaints despite normal lung tests
- Recent research shows hEDS patients have significantly lower accuracy in perceiving their lung volume compared to healthy individuals
- Mental health factors, particularly depression, strongly correlate with breathing difficulties in hEDS patients
- Researchers found hEDS patients exhibit erratic breathing patterns during cognitive tasks due to proprioceptive deficits
The Connection Between Hypermobility and Breathing Difficulties
Ehlers-Danlos syndromes (EDS) are a group of connective tissue disorders characterized by joint hypermobility, skin hyperextensibility, and tissue fragility. Hypermobile Ehlers-Danlos syndrome (hEDS) is the most common subtype, affecting not just joints but potentially all systems supported by connective tissue. While joint symptoms like pain and instability are well-recognized in hEDS, the impact on respiratory function has received less attention despite being a significant concern for many patients. Recent studies have begun to uncover why people with hypermobility often struggle with breathing issues even when traditional pulmonary function tests appear normal.
Scientists now believe that the same connective tissue anomalies affecting joints in hEDS also impact the thoracic cage and respiratory muscles. The structural changes in tissues involved in ventilation directly affect breathing mechanics. Additionally, proprioception—the body’s ability to sense position and movement—appears to play a critical role in respiratory function. People with hEDS often have impaired proprioception in their joints, and new research suggests this deficit extends to their ability to perceive lung volume accurately.
Proprioception and Lung Volume Perception
A groundbreaking study published in Scientific Reports investigated whether impaired proprioception affects lung volume perception and breathing control in hEDS patients. Researchers developed a specialized device to measure how accurately participants could perceive their lung volume during breathing exercises. The results were revealing: hEDS patients showed significantly lower accuracy in lung volume perception at 30% of inspired vital capacity compared to healthy control subjects. This finding provides the first objective evidence of a physiological basis for the breathing difficulties many hEDS patients report, despite normal results on standard pulmonary function tests.
The study also found that hEDS patients exhibited erratic ventilation patterns during cognitive tasks. When asked to perform mental activities while breathing, their breathing became more variable and less controlled than that of healthy individuals. This suggests that proprioceptive deficits influence ventilatory control in hEDS patients, especially when attention is divided. For many people with hEDS, this could explain why breathing feels more difficult during everyday activities that require mental focus.
Mental Health and Breathing Complaints
The relationship between hypermobility, breathing difficulties, and mental health appears increasingly significant. A cross-sectional study published in the International Journal of Environmental Research and Public Health found that most adults with hEDS (84.9%) report functional respiratory complaints, including changes in breathing patterns, chest pain, dizzy spells, shortness of breath, and chest tightness. Using the Nijmegen questionnaire to assess symptoms, researchers discovered strong correlations between breathing problems, central sensitization, disease perception, and mood disorders.
Breathing issues in hEDS patients linked to poorer mental health
Depression emerged as the most significant factor contributing to the severity of functional respiratory complaints in hEDS patients. Researchers identified three distinct clusters of patients: those with no functional respiratory complaints, those with mild complaints, and those with severe complaints. The severity of depression strongly predicted which group patients fell into, suggesting that addressing mental health should be an integral part of respiratory symptom management in hEDS. Additionally, 54.3% of study participants had probable anxiety, highlighting the complex interplay between physical symptoms and psychological well-being.
Clinical Implications and Future Directions
The emerging research on respiratory function in hypermobility syndromes has important implications for clinical care. Traditional pulmonary function tests may not capture the breathing difficulties experienced by people with hEDS, potentially leading to dismissal of legitimate symptoms. Healthcare providers should consider proprioceptive deficits and mental health factors when evaluating respiratory complaints in hypermobile patients. Specialized assessment tools that measure lung volume perception may prove more valuable than standard tests in this population.
Future treatment approaches may need to address both physical and psychological aspects of breathing difficulties in hEDS. Targeted proprioceptive training for respiratory muscles, breathing retraining techniques, and integrated mental health support could offer more effective management strategies. As research continues to uncover the complex relationships between hypermobility, proprioception, breathing mechanics, and mental health, people with hEDS and related conditions may finally find validation and more effective treatment for their long-misunderstood respiratory symptoms.
