Abstract
The objective of this study was to define pathologic mechanisms of primary spontaneous pneumothorax in patients with connective tissue dysplasia (CTD). Methods. We followed-up 42 young patients with CTD and bullous emphysema in the lung CT without history of primary spontaneous pneumothorax. Results of clinical, functional, radiological and molecular testing were compared with morphological examination of resected lung tissue specimens from 165 patients underwent lung surgery for primary spontaneous pneumothorax. Results. Two mechanisms underlying emphysema in CTD patients were defined. Conclusion: The results supported a hypothesis of causative relationship between congenital CTD and bullous emphysema in young patients.
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