Source: International Congress 2014 – ILDs 3
Disease area: Interstitial lung diseases

Abstract

LAM is a rare pulmonary cystic disease that may be sporadic (S-LAM) or associated with Tuberous Sclerosis Complex (TSC-LAM). This study was aimed at evaluating potential differences between TSC patients with or without LAM and between S-LAM and TSC-LAM patients. We analyzed 89 women (37±12 years, mean±DS), 11 affected by S-LAM and 78 by TSC. Among women affected by TSC (38±12 years), 27 had LAM (35%). These patients had a mild ventilatory defect, with a mean FEV1 almost normal (96±26% vs. 98±11%in TSC patients without LAM). TSC women with LAM were older (44±11 years) than those without (34±11 years, p<0.001). The MRC score (Medical Research Council) was 0.5±1.0 vs. 0.3±0.7 for TSC patients, with and without LAM, respectively (p=0.176). Patients affected by TSC-LAM had more air trapping and reduction of DLCO, but the difference was not statistically significant (p=0.404 and 0.068, respectively). In patients affected by TSC-LAM we found a higher rate of hepatic angiomyolipomas, compared with TSC patients without LAM (48% vs. 22%, p=0.032). No other differences associated with LAM emerged in TSC patients, such as genetic mutations, renal angiomyolipomas, skin lesions, epilepsy, encephalic lesions. S-LAM patients had a mean age of 42±9 years and 50% of these had a clinical history of at least a pneumothorax. Mean FEV1, DLCO/VA and RV/TLC were 76±27%, 61±19% and 151±29%, respectively. Dyspnea assessed with MRC scale was 2.3±1.6. As a whole, our TSC-LAM patients showed milder respiratory manifestations than S-LAM patients, probably because in TSC a screening to detect LAM is usually performed.

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G. Imeri, F. Di Marco, G. Palumbo, S. Terraneo, S. Centanni, F. Ribolla, G. F. Sferrazza Papa, G. Pellegrino, E. Rancati, E. M. Parazzini (Milan, Italy). Sporadic and TSC-related lymphangioleiomyomatosis (LAM): The experience of Ospedale San Paolo of Milan (preliminary data). Eur Respir J 2014; 44: Suppl. 58, 773

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