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Distinguishing the histological and radiological features of cystic lung disease in Birt-Hogg-Dubé syndrome from those of tobacco-related spontaneous pneumothorax.
Histopathology. 2014 Apr; 64(5):741-9.H

Abstract

AIMS

Birt-Hogg-Dubé syndrome (BHD) is a rare autosomal dominantly inherited genodermatosis that predisposes to cystic lung disease, leading to spontaneous pneumothoraces. This retrospective analysis of five BHD cases (two men, three women) compared lung histology and computed tomography (CT) imaging to a matched cohort of non-BHD patients with spontaneous pneumothoraces (SPN).

METHODS AND RESULTS

Lung was sampled during pleurodesis to resect bullae. Recurrent pneumothoraces was seen in two patients. Fourteen sets of histological slides (seven in each group) and 10 CT scans (five in each group) were reviewed. CT scans in BHD showed multiple cysts with a basal predominance and intraparenchymal/peribronchial distribution. On histological examination, BHD lungs showed punch-out cysts with no inflammation, and lacked subpleural fibroelastotic scars and smoking changes. In contrast, all SPN cases showed respiratory bronchiolitis and subpleural fibroelastotic scars.

CONCLUSIONS

This study emphasizes the importance of smoking history and topography of the lesions in assessing cystic lung disease. Pathologists need to remain alert to the possibility of BHD in the setting of recurrent pneumothoraces in a non-smoker, in particular in a woman, at any age, and should take part in a multidisciplinary approach to the diagnosis of cystic lung disease to obtain clinical and CT scan details.

Authors+Show Affiliations

Department of Histopathology, St Vincent's University Hospital, Dublin, Ireland.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Case Reports
Comparative Study
Journal Article

Language

eng

PubMed ID

24168179

Citation

Fabre, Aurelie, et al. "Distinguishing the Histological and Radiological Features of Cystic Lung Disease in Birt-Hogg-Dubé Syndrome From Those of Tobacco-related Spontaneous Pneumothorax." Histopathology, vol. 64, no. 5, 2014, pp. 741-9.
Fabre A, Borie R, Debray MP, et al. Distinguishing the histological and radiological features of cystic lung disease in Birt-Hogg-Dubé syndrome from those of tobacco-related spontaneous pneumothorax. Histopathology. 2014;64(5):741-9.
Fabre, A., Borie, R., Debray, M. P., Crestani, B., & Danel, C. (2014). Distinguishing the histological and radiological features of cystic lung disease in Birt-Hogg-Dubé syndrome from those of tobacco-related spontaneous pneumothorax. Histopathology, 64(5), 741-9. https://doi.org/10.1111/his.12318
Fabre A, et al. Distinguishing the Histological and Radiological Features of Cystic Lung Disease in Birt-Hogg-Dubé Syndrome From Those of Tobacco-related Spontaneous Pneumothorax. Histopathology. 2014;64(5):741-9. PubMed PMID: 24168179.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Distinguishing the histological and radiological features of cystic lung disease in Birt-Hogg-Dubé syndrome from those of tobacco-related spontaneous pneumothorax. AU - Fabre,Aurelie, AU - Borie,Raphael, AU - Debray,Marie Pierre, AU - Crestani,Bruno, AU - Danel,Claire, Y1 - 2013/12/26/ PY - 2013/07/23/received PY - 2013/10/24/accepted PY - 2013/10/31/entrez PY - 2013/10/31/pubmed PY - 2015/5/12/medline KW - Birt-Hogg-Dubé syndrome KW - computed tomography thorax KW - cysts KW - lung KW - pathology KW - tobacco SP - 741 EP - 9 JF - Histopathology JO - Histopathology VL - 64 IS - 5 N2 - AIMS: Birt-Hogg-Dubé syndrome (BHD) is a rare autosomal dominantly inherited genodermatosis that predisposes to cystic lung disease, leading to spontaneous pneumothoraces. This retrospective analysis of five BHD cases (two men, three women) compared lung histology and computed tomography (CT) imaging to a matched cohort of non-BHD patients with spontaneous pneumothoraces (SPN). METHODS AND RESULTS: Lung was sampled during pleurodesis to resect bullae. Recurrent pneumothoraces was seen in two patients. Fourteen sets of histological slides (seven in each group) and 10 CT scans (five in each group) were reviewed. CT scans in BHD showed multiple cysts with a basal predominance and intraparenchymal/peribronchial distribution. On histological examination, BHD lungs showed punch-out cysts with no inflammation, and lacked subpleural fibroelastotic scars and smoking changes. In contrast, all SPN cases showed respiratory bronchiolitis and subpleural fibroelastotic scars. CONCLUSIONS: This study emphasizes the importance of smoking history and topography of the lesions in assessing cystic lung disease. Pathologists need to remain alert to the possibility of BHD in the setting of recurrent pneumothoraces in a non-smoker, in particular in a woman, at any age, and should take part in a multidisciplinary approach to the diagnosis of cystic lung disease to obtain clinical and CT scan details. SN - 1365-2559 UR - https://www.unboundmedicine.com/medline/citation/24168179/Distinguishing_the_histological_and_radiological_features_of_cystic_lung_disease_in_Birt_Hogg_Dubé_syndrome_from_those_of_tobacco_related_spontaneous_pneumothorax_ L2 - https://doi.org/10.1111/his.12318 DB - PRIME DP - Unbound Medicine ER -