Modern treatment of patients with advanced lung disease includes not only pulmonary therapy but also integrative treatment of co-morbidities directly or indirectly linked to the underlying lung disease. Moreover, impairment of heath related quality of life is frequent in these patients and influences their physical and psychological "well-being". Therefore, a multidisciplinary team approach gives the opportunity to appropriately deal with all these issues. It has been shown that treating patients with advanced lung disease in specialised centres has a positive impact on survival, especially for patients with CF and IPF. Referral to the intensive care unit is not generally contraindicated and mortality depends on the underlying lung disease and co-morbidities. Nowadays, lung transplantation is a valuable therapeutic option in advanced lung disease and should be considered early especially in IPF patients.