Due to its characteristic brain tumors causing intellectual delay and seizures, TSC-LAM usually presents in childhood. Seibert et al report a series of 79 adult women with TSC-LAM, 45 of whom were diagnosed in adulthood. 15 did not meet diagnostic criteria until after age 18. Ann Intern Med 2011;154:806-813.
Lung vasculitis and alveolar hemorrhage. Ten articles in 103 pages. Semin Resp Crit Care Med 2011;32:243-346. Review.
Taveira-DaSilva reports an observational series of 19 women treated with sirolimus for lymphangioleiomyomatosis. Of 12 women with chylous pleural effusions, most had a large improvement or resolution in their effusions. Ann Intern Med 2011;154:797-805. In a randomized trial of 89 LAM patients published in NEJM March 2011, sirolimus reduced decline of lung function.
Toward a better diagnosis of idiopathic pulmonary fibrosis. Valeyre D, Eur Resp Rev 2011;20:108-113.
Analyzing six minute walk distances from 822 people with idiopathic pulmonary fibrosis in an interferon-gamma-1b clinical trial, du Bois et al found those whose decline in six minute walk test distance was >50 meters in 24 weeks had a four-fold risk of death within one year. In everyone but those with the very worst functional status, 6MWT [… read more]
Diffuse parenchymal lung disease, Update for 2010: Markart P et al, AJRCCM 2011;183:1316-1321.
Cryptogenic and secondary organizing pneumonia: Drakopanagiotakis F et al, CHEST 2011;139:893-900. COP and BOOP review.
Transtracheal oxygen therapy: Christopher KL, Schwartz MD
Lymphangioleiomyomatosis. Harari S, Eur Resp Review 2011;20:34-44. LAM review.
Pulmonary Sarcoidosis: Concise Clinical Review. Baughman RP, AJRCCM 2011;183:573-581.
Clinical Course & Prediction of Survival in Idiopathic Pulmonary Fibrosis: Ley B, AJRCCM 2011;183:431-440.
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]