TABLE 2.
LAM | PLCH | |
Related to smoking | Not evident | Strong |
Gender predilection | Yes (female) | No |
Genetic mutations | TSC1 or more frequently, TSC2 | BRAFV600E, NRAS, MAP2K1 |
HRCT pattern | Cystic | Nodular-cystic Cystic Nodular |
Multisystem involvement | Frequent | Possible |
Treatment | Sirolimus# | Smoking cessation Steroids Vinblastine Possible role of cladribrine and BRAF inhibitors |
#: approved by the US Food and Drug Administration in 2015, recognised as orphan drug by the European Medicines Agency in 2016. HRCT: high-resolution computed tomography.