RT Journal Article
SR Electronic
T1 Rate of FEV1 decline in lymphangioleiomyomatosis associated with tuberous sclerosis complex
JF European Respiratory Journal
JO Eur Respir J
FD European Respiratory Society
SP P683
VO 40
IS Suppl 56
A1 Romain Lazor
A1 Martine Reynaud-Gaubert
A1 Vincent Cottin
A1 Jacques Lacronique
A1 Camille Taillé
A1 Benoit Wallaert
A1 Sandrine Hirschi
A1 Bruno Crestani
A1 Jean-François Cordier
A1 the Groupe d Etudes et de Recherche sur les Maladies Orphelines Pulmonaires
YR 2012
UL http://erj.ersjournals.com/content/40/Suppl_56/P683.abstract
AB Pulmonary lymphangioleiomyomatosis (LAM) is a rare disease characterized by proliferation of abnormal TSC1/2-mutated smooth muscle cells in the lung parenchyma, leading to progressive cystic destruction, lung function decline, and respiratory insufficiency. LAM may be either sporadic (S-LAM) or associated with the tuberous sclerosis complex (TSC-LAM). Cross-sectional studies have suggested that TSC-LAM is less severe than S-LAM, but no longitudinal data are available. We retrospectively compared the rate of FEV1 decline in 16 patients with TSC-LAM and 53 patients with S-LAM diagnosed according to ERS 2010 criteria with a lung function follow-up ≥1 year. Results are shown below.View this table:Conclusion: Although diagnosed at an earlier age than S-LAM, patients with TSC-LAM had similar mean FEV1 at diagnosis, and similar mean rate of FEV1 decline. Lung function follow-up similar to S-LAM may be recommended in TSC-LAM.