Lymphangioleiomyomatosis. Harari S, Eur Resp Review 2011;20:34-44. LAM review.
Song et al retrospectively reviewed 461 cases of IPF (269 biopsy-proven) in Korea. More than a third of patients were hospitalized for respiratory deteriorations over a 2-year period. About half the deteriorations were attributed to acute exacerbations of IPF, with most of the rest attributed to infections (half the patients had BAL and/or ET aspirates [… read more]
McCormack et al randomized 89 women with lymphangioleiomyomatosis to receive 2 mg rapamycin or placebo daily for 12 months, followed by 12 months of drug-free observation. Women receiving sirolimus had stable lung function on spirometry, fewer symptoms, fewer “respiratory events,” and better quality of life during the treatment period. This was tempered by GI, dermatologic, [… read more]