@article {LazorP683, author = {Romain Lazor and Martine Reynaud-Gaubert and Vincent Cottin and Jacques Lacronique and Camille Taill{\'e} and Benoit Wallaert and Sandrine Hirschi and Bruno Crestani and Jean-Fran{\c c}ois Cordier and the Groupe d Etudes et de Recherche sur les Maladies Orphelines Pulmonaires}, title = {Rate of FEV1 decline in lymphangioleiomyomatosis associated with tuberous sclerosis complex}, volume = {40}, number = {Suppl 56}, elocation-id = {P683}, year = {2012}, publisher = {European Respiratory Society}, abstract = {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.}, issn = {0903-1936}, URL = {https://erj.ersjournals.com/content/40/Suppl_56/P683}, eprint = {https://erj.ersjournals.com/content/40/Suppl_56/P683.full.pdf}, journal = {European Respiratory Journal} }