title: [A rare form of obstructive pulmonary disease].
abstract: 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.
authors.affiliation: Erasmus MC, Rotterdam.
authors.forename: C J
authors.initials: CJ
authors.lastname: van Loenhout
authors.forename: M A
authors.initials: MA
authors.lastname: den Bakker
authors.forename: M S
authors.initials: MS
authors.lastname: van Wijsenbeek
authors.forename: R A S
authors.initials: RA
authors.lastname: Hoek
authors.forename: P Th W
authors.initials: PT
authors.lastname: van Hal
mesh_terms: D000328:Adult; D001706:Biopsy; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D008168:Lung / Q000000981:diagnostic imaging*; D008173:Lung Diseases, Obstructive / Q000175:diagnosis / Q000209:etiology*; D008175:Lung Neoplasms / Q000150:complications* / Q000175:diagnosis; D018192:Lymphangioleiomyomatosis / Q000150:complications* / Q000175:diagnosis; D012129:Respiratory Function Tests; D014057:Tomography, X-Ray Computed