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Author Notes:

Correspondence and requests for reprints should be addressed to Nishant Gupta, M.D., Division of Pulmonary, Critical Care and Sleep Medicine, Department of Internal Medicine, College of Medicine, University of Cincinnati, 231 Albert Sabin Way, MSB Room 6053, ML 0564, Cincinnati, OH 45267. E-mail: guptans@ucmail.uc.edu

Author Contributions: N.G.: had full access to all of the data in the study and takes responsibility for the integrity of the data and the accuracy of the data analysis; N.G., E.J.K., E.P.H., and F.X.M.: devised the study questionnaire; S.E.-C., S.V., and M.G.D.: helped in patient recruitment; N.G. and L.E.J.: performed the data analysis; and all authors: contributed substantially to the writing and editing of the manuscript.

See supplemental materials for disclosure statement.

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Research Funding:

Supported by National Institutes of Health grants U54HL127672; and 1 UL1TR001425-01.

Keywords:

  • Science & Technology
  • Life Sciences & Biomedicine
  • Respiratory System
  • pneumothorax
  • pleurodesis
  • air travel
  • Birt-Hogg-Dube syndrome
  • CYSTIC LUNG-DISEASES
  • PULMONARY CYSTS
  • AIR-TRAVEL
  • COMPUTED-TOMOGRAPHY
  • LYMPHANGIOLEIOMYOMATOSIS
  • FAMILIES
  • CHEST
  • RISK

Spontaneous Pneumothoraces in Patients with Birt-Hogg-Dube Syndrome

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Journal Title:

Annals of the American Thoracic Society

Volume:

Volume 14, Number 5

Publisher:

, Pages 706-713

Type of Work:

Article | Final Publisher PDF

Abstract:

Rationale: Spontaneous pneumothorax is a common complication of Birt-Hogg-Dube syndrome (BHD). Objectives: The optimal approach to treatment and prevention of BHD-associated spontaneous pneumothorax, and to advising patients with BHD regarding risk of pneumothorax associated with air travel, is not well established. Methods: Patients with BHD were recruited from the Rare Lung Diseases Clinic Network and the BHD Foundation and surveyed about disease manifestations and air travel experiences. Results: A total of 104 patients completed the survey. The average age at diagnosis was 47 years, with an average delay from first symptoms of 13 years. Pulmonary cysts were the most frequent phenotypic manifestation of BHD, present in 85% of patients. Spontaneous pneumothorax was the presenting manifestation that led to the diagnosis of BHD in 65% of patients, typically after the second episode (mean, 2.4 episodes). Seventy-nine (76%) of 104 patients had at least one spontaneous pneumothorax during their lifetime, and 82% had multiple pneumothoraces. Among patients with multiple pneumothoraces, 73% had an ipsilateral recurrence, and 48% had a subsequent contralateral spontaneous pneumothorax following a sentinel event. The mean ages at first and second pneumothoraces were 36.5 years (range, 14-63 yr) and 37 years (range, 20-55 yr), respectively. The average number of spontaneous pneumothoraces experienced by patients with a sentinel pneumothorax was 3.6. Pleurodesis was generally performed after the second (mean, 2.4) ipsilateral pneumothorax and reduced the ipsilateral recurrence rate by half. A total of 11 episodes of spontaneous pneumothorax occurred among eight patients either du ring or within the 24-hour period following air travel, consistent with an air travel-related pneumothorax rate of 8% per patient and 0.12% per flight. Prior pleurodesis reduced the occurrence of a subsequent flight-related pneumothorax. Conclusions: Spontaneous pneumothorax is an important, recurrent manifestation of pulmonary involvement in patients with BHD, and pleurodesis should be considered following the initial pneumothorax to reduce the risk of recurrent episodes. In general, in patients with BHD, pneumothorax occurs in about 1-2 per 1,000 flights, and the risk is lower among patients with a history of prior pleurodesis.

Copyright information:

© 2017 by the American Thoracic Society.

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