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Enlarged Dural Sac in Idiopathic Bronchiectasis Implicates Heritable Connective Tissue Gene Variants
M Leigh Anne Daniels1, Katherine R Birchard2, Jared R Lowe3
11 Department of Medicine and.
Annals of the American Thoracic Society
|July 14, 2016
Summary
Patients with idiopathic bronchiectasis have larger dural sacs, linked to lung infections and long fingers. This suggests a genetic connection to connective tissue disorders.
Area of Science:
- Medical imaging
- Pulmonology
- Genetics
Background:
- Idiopathic bronchiectasis often affects females with asthenic body types.
- Patients frequently experience nontuberculous mycobacterial infections.
- Phenotypic features resemble heritable connective tissue disorders.
Purpose of the Study:
- To compare lumbar dural sac size in idiopathic bronchiectasis patients versus controls.
- To investigate correlations between dural sac size and connective tissue disorder-like features.
Main Methods:
- Magnetic resonance imaging (MRI) of L1-L5 was used to measure dural sac diameter.
- 71 idiopathic bronchiectasis patients, 72 controls, 29 cystic fibrosis, and 24 Marfan syndrome patients were included.
- Blinded readers performed measurements; statistical analyses compared groups and associations.
Main Results:
- Idiopathic bronchiectasis patients showed significantly larger dural sac diameters than controls and cystic fibrosis patients.
- Increased dural sac size correlated with pulmonary nontuberculous mycobacterial infection and long fingers.
- A trend toward larger dural sacs was observed in patients with scoliosis and a family history.
Conclusions:
- Idiopathic bronchiectasis is associated with an enlarged dural sac.
- This finding supports a genetic link involving heritable connective tissue and TGF-β pathways.
- Dural sac size may serve as a potential biomarker in idiopathic bronchiectasis.
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