A Study of Nintedanib for LymphAngioleioMyomatosis (LAM)

Inclusion Criteria: * Written Informed Consent for participating to trial. * Patient aged ≥ 18 years at visit 1. * Sporadic or TSC associated LAM, classified as ''definite'' by the European Respiratory Society criteria and /or serum VEGFD level \>/= 800 mg/ml, and evidence of a 10% deterioration in FEV1 and /or loss of 80 ml of FEV1 or more in the last year (post bronchodilator). Also LAM patients with proven side effects and/or toxicities/ contraindications to sirolimus therapy will be eligible for this study. Exclusion Criteria: Laboratory parameters have to satisfy entry criteria as shown below: * Laboratory parameters (screening) * AST, ALT \> 1.5 x ULN * Bilirubin \> 1.5 x ULN * Positivity for HIV or Hepatitis. * Chylous effusions. * Relapsing pneumothorax. * Angiomyolipoma \> 5 cm. * Treatment with mTOR inhibitors in the last month. * Patient eligible for Lung Transplantation. * Hormone therapy. * Patients are excluded if they are post lung transplant or had previously been diagnosed with a pneumothorax, chylous effusion, bleeding angiomyolipoma within the previous 6 months. * Current smokers. * Other diseases: * Cardiac disease. * Myocardial infarction within 6 months of visit 2. * Unstable angina within 1 month of visit 2. * Bleeding Risk: * Known genetic predisposition to bleeding. * Patients who require fibrinolysis, full-dose therapeutic anticoagulation (e.g. vitamin K antagonists, heparin, NOA) or high dose antiplatelet therapy2. * History of ha…