TY - JOUR T1 - Severe idiopathic pulmonary fibrosis: what can be done? JF - European Respiratory Review JO - EUROPEAN RESPIRATORY REVIEW DO - 10.1183/16000617.0047-2017 VL - 26 IS - 145 SP - 170047 AU - Antonella Caminati AU - Roberto Cassandro AU - Olga Torre AU - Sergio Harari Y1 - 2017/09/30 UR - http://err.ersjournals.com/content/26/145/170047.abstract N2 - 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 ER -