C. Kingswood and the Review Panel of the ERS LAM Task Force

ymphangioleiomyomatosis (LAM) is a rare lung disease, which occurs sporadically or in association with the genetic disease tuberous sclerosis complex (TSC) [1, 2]. Sporadic LAM affects,1 in 400,000 adult females; in TSC, LAM occurs in 30–40 % of adult females [3, 4] and exceptionally in males and children [5, 6]. Patients with LAM usually develop progressive dyspnoea and recurrent pneumothorax, chylous collections and occasional haemoptysis [1]. Extra pulmonary lymphadenopathy and cystic masses of the axial lymphatics termed lymphangioleio-myomas can result in abdominal and pelvic lymphatic obstruction [7]. LAM is often asso-ciated with angiomyolipoma in the kidneys [8]