RT Journal Article SR Electronic T1 Severe idiopathic pulmonary fibrosis: what can be done? JF European Respiratory Review JO EUROPEAN RESPIRATORY REVIEW FD European Respiratory Society SP 170047 DO 10.1183/16000617.0047-2017 VO 26 IS 145 A1 Antonella Caminati A1 Roberto Cassandro A1 Olga Torre A1 Sergio Harari YR 2017 UL http://err.ersjournals.com/content/26/145/170047.abstract AB Idiopathic pulmonary fibrosis (IPF) remains a challenging disease to manage. Two drugs are now available that can slow disease progression in patients with mild-to-moderate IPF. This means that early diagnosis is mandatory, because there are no proven effective therapies for severe IPF. This lack of proven therapies may be at least partially due to the fact that severe IPF patients are usually not enrolled in randomised, prospective, multicentre, international trials. Clinical observation experiences and preliminary results of long-term, open-label extensions of clinical trials suggest that both pirfenidone and nintedanib may also slow or decrease progression in patients with severe IPF. However, data are sparse and obtained from a relatively small number of patients. Lung transplantation should be taken into account early and discussed with patients, when indicated. Rehabilitative strategies are important and effective supportive therapies. The needs of patients with severe IPF are similar to those of patients with an advanced neoplastic disease. Palliative care and psychological support play an important role in the relief of symptoms of anxiety and depression. Accordingly, these therapeutic approaches should start early in IPF patients.Patients with severe IPF require a global approach similar to patients with advanced cancer disease http://ow.ly/1apy30dYRjU