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A New LAM Clinical Practice Guideline

July 1, 2017 at 9:50 am

By David Lederer, MD, MS, editor, Annals of the American Thoracic Society

Follow Dr. Lederer on Twitter @AnnalsATS

Summary for Clinicians: Lymphangioleiomyomatosis Diagnosis and Management Clinical Practice Guideline

In the July Annals of the American Thoracic Society, the ATS and Japanese Respiratory Society published the following recommendations addressing the use of vascular endothelial growth factor D (VEGF-D) as a diagnostic test and the therapeutic role of sirolimus, doxycycline and hormonal manipulation in patients with lymphangioleiomyomatosis:

  • For selected patients with LAM with problematic chylous effusions, we suggest treatment with sirolimus before invasive management.
  • We suggest NOT using doxycycline as treatment for LAM.
  • We suggest NOT using hormonal therapy as treatment for LAM.
  • For patients whose computed tomography scan shows cystic abnormalities characteristic of LAM, but who have no other confirmatory clinical or extrapulmonary radiologic features of LAM, we recommend vascular endothelial growth factor D testing to establish the diagnosis of LAM before consideration of proceeding to diagnostic lung biopsy.

 

July Highlights

An Official American Thoracic Society Workshop Report: Noninvasive Identification of Inspiratory Flow Limitation in Sleep Studies

Care of the Critically Ill Burn Patient: An Overview from the Perspective of Optimizing Palliative Care

Mortality after Respiratory Isolation of Nontuberculous Mycobacteria: A Comparison of Patients Who Did and Did Not Meet Disease Criteria

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