Cystic lung disease in Birt-Hogg-Dubé syndrome

Dereje S. Ayo, Gregory L. Aughenbaugh, Eunhee S. Yi, Jennifer L. Hand, Jay H Ryu

Research output: Contribution to journalArticle

65 Citations (Scopus)

Abstract

Ring in patients with Birt-Hogg-Dubé (BHD) syndrome, a rare, inheritable, multisystem disorder. Methods: We retrospectively reviewed five patients with BHD syndrome evaluated at the Mayo Clinic Rochester from 1998 through 2005. Results: Mean age (± SD) at the time of pulmonary evaluation was 56.4 ± 4.8 years; four patients were men. Three patients had not received a diagnosis of BHD syndrome at the time of initial CT of the chest. Three patients had a smoking history, and two were nonsmokers. Two patients had a history of recurrent pneumothoraces. Pulmonary function tests available in four patients revealed normal results in one patient and mild airflow obstruction or nonspecific pattern of abnormalities in three patients. CT of the chest revealed cystic lung disease in all five patients; cysts were round to oval in shape, ranged widely in size, and were randomly distributed throughout the lungs, except for a predilection to involve the lung bases more extensively. Three patients with a smoking history had more severe cystic changes compared to nonsmokers and included both patients with recurrent pneumothoraces. Surgical lung biopsy available in one patient revealed emphysema-like changes. Follow-up CT scans available in four patients revealed relative stability over a median interval of 20 months (range, 3 to 66 months). Conclusion: We conclude that cystic lung disease in BHD syndrome varies widely in severity, mimics pulmonary lymphangioleiomyomatosis, and may be worsened by smoking.

Original languageEnglish (US)
Pages (from-to)679-684
Number of pages6
JournalChest
Volume132
Issue number2
DOIs
StatePublished - Aug 2007

Fingerprint

Lung
Smoking
Pneumothorax
Cystic Disease Of Lung
Thorax
History
Lymphangioleiomyomatosis
Respiratory Function Tests
Emphysema
Cysts
Biopsy

Keywords

  • Birt-Hogg-Dubé
  • CT scan pulmonary
  • Cystic lung disease
  • Fibrofolliculoma
  • Pneumothorax
  • Smoking
  • Syndrome

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine

Cite this

Ayo, D. S., Aughenbaugh, G. L., Yi, E. S., Hand, J. L., & Ryu, J. H. (2007). Cystic lung disease in Birt-Hogg-Dubé syndrome. Chest, 132(2), 679-684. https://doi.org/10.1378/chest.07-0042

Cystic lung disease in Birt-Hogg-Dubé syndrome. / Ayo, Dereje S.; Aughenbaugh, Gregory L.; Yi, Eunhee S.; Hand, Jennifer L.; Ryu, Jay H.

In: Chest, Vol. 132, No. 2, 08.2007, p. 679-684.

Research output: Contribution to journalArticle

Ayo, DS, Aughenbaugh, GL, Yi, ES, Hand, JL & Ryu, JH 2007, 'Cystic lung disease in Birt-Hogg-Dubé syndrome', Chest, vol. 132, no. 2, pp. 679-684. https://doi.org/10.1378/chest.07-0042
Ayo DS, Aughenbaugh GL, Yi ES, Hand JL, Ryu JH. Cystic lung disease in Birt-Hogg-Dubé syndrome. Chest. 2007 Aug;132(2):679-684. https://doi.org/10.1378/chest.07-0042
Ayo, Dereje S. ; Aughenbaugh, Gregory L. ; Yi, Eunhee S. ; Hand, Jennifer L. ; Ryu, Jay H. / Cystic lung disease in Birt-Hogg-Dubé syndrome. In: Chest. 2007 ; Vol. 132, No. 2. pp. 679-684.
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