Evidence-based recommendations for the diagnosis and treatment of patients with LAM are provided. Frequent reassessment and updating will be needed.

only one of the studies (6). Thus, the proposed threshold serum level is based on the results obtained by a single laboratory. Although the fundamentals of LAM have been extensively discussed, with special regard to therapeutic issues, other more general aspects concerning the practical management of the disease should be considered, such as use of bronchodilators, pulmonary rehabilitation, and indications for lung transplant. Pregnancy and air travel limitation are two further matters of great importance for the general pulmonologist and should be discussed and dealt with separately. n Author disclosures are available with the text of this letter at www.atsjournals.org.