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Cystic lung disease in Birt-Hogg-Dube syndrome.
Chest. 2007 Aug; 132(2):679-84.Chest

Abstract

BACKGROUND

To describe the clinical, radiologic, and histopathologic aspects of cystic lung disease occurring 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.

Authors+Show Affiliations

Division of Pulmonary and Critical Care Medicine, Desk East 18, Mayo Clinic, 200 First St SW, Rochester, MN 55905, USA.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article

Language

eng

PubMed ID

17505035

Citation

Ayo, Dereje S., et al. "Cystic Lung Disease in Birt-Hogg-Dube Syndrome." Chest, vol. 132, no. 2, 2007, pp. 679-84.
Ayo DS, Aughenbaugh GL, Yi ES, et al. Cystic lung disease in Birt-Hogg-Dube syndrome. Chest. 2007;132(2):679-84.
Ayo, D. S., Aughenbaugh, G. L., Yi, E. S., Hand, J. L., & Ryu, J. H. (2007). Cystic lung disease in Birt-Hogg-Dube syndrome. Chest, 132(2), 679-84.
Ayo DS, et al. Cystic Lung Disease in Birt-Hogg-Dube Syndrome. Chest. 2007;132(2):679-84. PubMed PMID: 17505035.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Cystic lung disease in Birt-Hogg-Dube syndrome. AU - Ayo,Dereje S, AU - Aughenbaugh,Gregory L, AU - Yi,Eunhee S, AU - Hand,Jennifer L, AU - Ryu,Jay H, Y1 - 2007/05/15/ PY - 2007/5/17/pubmed PY - 2007/10/3/medline PY - 2007/5/17/entrez SP - 679 EP - 84 JF - Chest JO - Chest VL - 132 IS - 2 N2 - BACKGROUND: To describe the clinical, radiologic, and histopathologic aspects of cystic lung disease occurring 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. SN - 0012-3692 UR - https://www.unboundmedicine.com/medline/citation/17505035/Cystic_lung_disease_in_Birt_Hogg_Dube_syndrome_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S0012-3692(15)37467-5 DB - PRIME DP - Unbound Medicine ER -