Frontiers of Medicine

, Volume 6, Issue 4, pp 395–405

Utility of transbronchial biopsy in the diagnosis of lymphangioleiomyomatosis

  • Riffat Meraj
  • Kathryn A. Wikenheiser-Brokamp
  • Lisa R. Young
  • Sue Byrnes
  • Francis X. McCormack
Research Article
  • 163 Downloads

Abstract

Pulmonary lymphangioleiomyomatosis (LAM) is a rare cystic lung disease that targets women during their reproductive years. A confident diagnosis can often be based on clinical grounds, but diagnostic certainty requires pathological analysis. Although surgical lung biopsy is considered the gold standard for obtaining tissue in patients with diffuse lung disease, it is also associated with higher morbidity and mortality than alternative, less invasive techniques. The objective of our study was to examine the utility of transbronchial biopsy in the diagnosis of LAM. We conducted two online surveys of over 1 000 LAM patients registered with the LAM Foundation who were accessible by email. Transbronchial biopsy specimens were subsequently collected and reviewed by an expert pathologist to validate the diagnosis. We found that transbronchial biopsy has a yield of approximately 60% in patients with LAM. We conclude that transbronchial biopsy may be a safe and effective method for establishing the diagnosis of LAM, obviating the need for surgical lung biopsy in more than half of LAM patients.

Keywords

lymphangioleiomyomatosis lymphangiomyomatosis multicystic lung disease diffuse cystic lung disease transbronchial biopsy perivascular epithelioid cell tumor (PEComa) HMB-45 

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

Copyright information

© Higher Education Press and Springer-Verlag Berlin Heidelberg 2012

Authors and Affiliations

  • Riffat Meraj
    • 1
  • Kathryn A. Wikenheiser-Brokamp
    • 2
  • Lisa R. Young
    • 3
  • Sue Byrnes
    • 4
  • Francis X. McCormack
    • 1
  1. 1.Division of Pulmonary, Critical Care and Sleep Medicine, Department of Internal MedicineUniversity of Cincinnati School of MedicineCincinnatiUSA
  2. 2.Pathology and Laboratory MedicineCincinnati Children’s Hospital Medical Center and University of Cincinnati School of MedicineCincinnatiUSA
  3. 3.Division of Pediatric Pulmonary Medicine, Department of PediatricsVanderbilt UniversityNashvilleUSA
  4. 4.The LAM FoundationCincinnatiUSA

Personalised recommendations