Hope is the Air We Breathe

New Guidelines for Early Diagnosis and Treatment of Lymphangioleiomyomatosis

Download the 2016 LAM Clinical Practice Guidelines here.
DOWNLOAD THE 2017 LAM CLINICAL PRACTICE GUIDELINES HERE. 

Lymphangioleiomyomatosis - or LAM - is a rare lung disease that usually strikes women during the prime of their lives. With the release of new clinical guidelines comes better understanding for the appropriate treatment of this debilitating illness, for both healthcare providers and patients alike. With this greater understanding comes greater hope for a cure. Just as air is vital to life, guidelines for diagnosis and treatment are vital to those living with LAM – and to the physicians working to treat them.

Top Things to Know:

  • LAM patients with abnormal or declining lung function should be treated with sirolimus.
  • LAM patients with problematic chylous effusions can begin sirolimus treatments prior to and/or as an alternative to invasive surgical management.
  • Doxycycline is not recommended as a treatment for LAM.
  • Hormonal therapy is not recommended as a treatment for LAM.
  • VEGF-D testing is recommended for patients whose CT scan shows cystic abnormalities characteristic of LAM, to assist with diagnosis.
  • For patients who have cystic changes on HRCT of the chest that are characteristic of LAM, but have no additional confirmatory features of LAM (i.e., clinical, radiologic, or serologic), HRCT alone is not recommended to make a clinical diagnosis of LAM.
  • When a definitive diagnosis is required in patients who have parenchymal cysts on HRCT that are characteristic of LAM, but no additional confirmatory features of LAM (i.e., clinical, radiologic, or serologic), a diagnostic approach that includes transbronchial lung biopsy before a surgical lung biopsy is recommended.
  • Patients with LAM should be offered ipsilateral pleurodesis after their initial pneumothorax rather than waiting for a recurrent pneumothorax before intervening with a pleural symphysis procedure.
  • Previous unilateral or bilateral pleural procedures (i.e., pleurodesis or pleurectomy) should not be considered a contraindication to lung transplantation in patients with LAM.