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.

The paper presents the study of 286 patients with spontaneous pneumotoraks. It was revealed that the patients with sings of CTD after similar methods of surgical treatment had more relapses and intermittent course of spontaneous pneumotoraks. We developed the models to predict the relapse rate of spontaneous pneumatoraks for the closest 12 month. Connective tissue dysplasia, surgical treatment with pleurodesis induction and history of smoking are the main predictors of model.

Summary. Results of observation of 642 patients with primary spontaneous pheumothorax are given in this article. To determine the etiology of bullous emphysema we performed targeted search of phenotypic criteria of inherited connective tissue disorders. We found that 75 % of patients with primary spontaneous pheumothorax had signs of the connective tissue dysplasia such as the asthenic somatotype, low body mass index, the chest and the spine deformities, joint hypermobility, mitral valve prolapse, myopia, varicose phlebectasia, etc. Results of the study confirm the fact that inherited disorders of the connective tissue could play an important role in the pathogenesis of bullous emphysema.