Asthma and airways collapse in two heritable disorders of connective tissue
Open accessAbstract
Objectives This study investigated the clinical impression that there was an increased prevalence of respiratory disorders in both the Hypermobility Syndrome (HMS)/Benign Joint Hypermobility Syndrome (BJHS) and Ehlers-Danlos Syndrome (EDS), compared with the normal population.Methods A questionnaire was distributed to 509 subjects (221 healthy controls, 126 HMS, 162 EDS) who documented respiratory symptoms and previously diagnosed respiratory and atopic disorders. A subgroup of 157 responders underwent full clinical and serological assessments, and 57 subjects were assessed physiologically.Results A significant increase in the frequency of a wide range of respiratory symptoms and reduced exercise tolerance was observed in subjects with both HMS and EDS compared with controls. In particular, there was an increased prevalence of asthmatic symptoms (HMS: OR 2.4, 95% CI 1.4-4.1, p = 0.002; EDS: OR 3.1, 95% CI 1.8-5.2, p<0.001) and atopy (HMS: OR 2.7, 95% CI 1.6-4.5, p<0.001; EDS: OR 2.6, 95% CI 1.6-4.4, p<0.001), which was subsequently confirmed by clinical assessment. Pulmonary physiological studies revealed increased lung volumes, impaired gas exchange and an increased tendency of both the lower and upper airways to collapse.Conclusions We have demonstrated, for the first time, that individuals with HMS/BJHS and EDS have respiratory symptoms in association with various pulmonary physiological abnormalities. The increased prevalence of asthma may be due to linkage disequilibrium between the genes causing these conditions or a function of the connective tissue defect itself. In the non-asthmatic population, changes in the mechanical properties of the bronchial airways and lung parenchyma may underlie the observed increased tendency of the airways to collapse.
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Clinical research described comorbidities in hEDS/HSD patients: functional gastrointestinal and eosinophilic disorders, increased prevalence of asthma, and chronic pain syndromes.
“Over the past few years, clinical research described the presence of comorbidities in hEDS/HSD patients, such as functional gastrointestinal and eosinophilic disorders [140,141], increased prevalence of asthma [142], and chronic pain syndromes, i.e., chronic fatigue, fibromyalgia, irritable bowel disease, and inflammatory joints conditions [111,143], though specific underlying causes and”
From Cellular and Molecular Mechanisms in the Pathogenesis of Classical, Vascular, and Hypermobile Ehlers‒Danlos Syndromes (2019) · cites this paper as [142]
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- Authors
- Morgan, A. W., Pearson, S. B., Davies, S., Gooi, H. C., & Bird, H. A.
- Affiliations
- Chapel Allerton Hospital (GB)
- Leeds General Infirmary (GB)
- St James's University Hospital (GB)
- Corresponding author
- Morgan, A W
- ORCID
- Venue
- Annals of the Rheumatic Diseases
- Publisher
- Elsevier BV
- Published
- 2007-10
- Volume
- 66
- Issue
- 10
- Pages
- 1369-1373
- ISSN
- 0003-4967 (ISSN-L)
- PMC
- PMC1994284
- OpenAlex
- W2031225542
- Cited by
- 68
- References
- 40
- Citations by year
- 2026: 5 · 2025: 3 · 2024: 4 · 2023: 8 · 2022: 5 · 2021: 5 · 2020: 3 · 2019: 4 · 2018: 4 · 2017: 6 · 2016: 4 · 2015: 2 · 2014: 3 · 2013: 6 · 2012: 2
- FWCI
- 0.36
- Type
- journal article
- Language
- en
- Open access
- green
- www.ncbi.nlm.nih.gov
- Locations
- Annals of the Rheumatic Diseases (publishedVersion)
- PubMed Central (publishedVersion)
- PubMed (publishedVersion)
- HighWire Press Open Archive (submittedVersion)
- Topics
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Connective tissue disorders research
- Health Sciences › Medicine › Physiology › Asthma and respiratory diseases
- Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Interstitial Lung Diseases and Idiopathic Pulmonary Fibrosis
- Keywords
- asthma, airway collapse, Ehlers-Danlos syndrome, benign joint hypermobility syndrome, hereditary connective tissue disorders, atopy, respiratory symptoms, exercise tolerance, lung volume, gas exchange impairment, linkage disequilibrium
- MeSH terms
- Adult, Asthma, Ehlers-Danlos Syndrome, Female, Humans, Joint Instability, Lung, Male, Middle Aged, Respiration Disorders, Respiratory Function Tests, Respiratory Hypersensitivity, Smoking, Syndrome
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