A rare form of obstructive pulmonary disease

Lymphangioleiomyomatosis (LAM) is characterised by progressive dyspnoea, spontaneous pneumothorax and cystic pulmonary destruction. The disease may show similarities with emphysema clinically, radiologically and on lung function tests. A 44-year-old woman was referred for lung transplantation becaus...

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Bibliographic Details
Published inNederlands tijdschrift voor geneeskunde Vol. 160; p. D757
Main Authors van Loenhout, C J, den Bakker, M A, van Wijsenbeek, M S, Hoek, R A S, van Hal, P Th W
Format Journal Article
LanguageDutch
Published Netherlands 2016
Subjects
Adult
Biopsy
Diagnosis, Differential
Female
Humans
Lung - diagnostic imaging
Lung Diseases, Obstructive - diagnosis
Lung Diseases, Obstructive - etiology
Lung Neoplasms - complications
Lung Neoplasms - diagnosis
Lymphangioleiomyomatosis - complications
Lymphangioleiomyomatosis - diagnosis
Respiratory Function Tests
Tomography, X-Ray Computed
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Summary:Lymphangioleiomyomatosis (LAM) is characterised by progressive dyspnoea, spontaneous pneumothorax and cystic pulmonary destruction. The disease may show similarities with emphysema clinically, radiologically and on lung function tests. A 44-year-old woman was referred for lung transplantation because of a 6-year history of dyspnoea and severe obstructive pulmonary function disorder with decreased diffusion capacity. Both her relatively young age and the fact that she had never smoked made us doubt the diagnosis 'COPD'. The pulmonary cysts seen on high-resolution CT (HRCT) suggested LAM. This was confirmed when we revised a pulmonary biopsy that had previously been performed. CT investigation should be carried out in patients with severe obstructive pulmonary disease without a risk profile appropriate for COPD. Diffuse, homogenous cysts on CT scan can indicate LAM, particularly in women. Conflict of interest and financial support: none declared.
ISSN:0028-2162
1876-8784