title: Clinical review: idiopathic pulmonary fibrosis acute exacerbations--unravelling Ariadne's thread.
abstract: Idiopathic pulmonary fibrosis (IPF) is a dreadful, chronic, and irreversibly progressive fibrosing disease leading to death in all patients affected, and IPF acute exacerbations constitute the most devastating complication during its clinical course. IPF exacerbations are subacute/acute, clinically significant deteriorations of unidentifiable cause that usually transform the slow and more or less steady disease decline to the unexpected appearance of acute lung injury/acute respiratory distress syndrome (ALI/ARDS) ending in death. The histological picture is that of diffuse alveolar damage (DAD), which is the tissue counterpart of ARDS, upon usual interstitial pneumonia, which is the tissue equivalent of IPF. ALI/ARDS and acute interstitial pneumonia share with IPF exacerbations the tissue damage pattern of DAD. 'Treatment' with high-dose corticosteroids with or without an immunosuppressant proved ineffective and represents the coup de grace for these patients. Provision of excellent supportive care and the search for and treatment of the 'underlying cause' remain the only options. IPF exacerbations require rapid decisions about when and whether to initiate mechanical support. Admission to an intensive care unit (ICU) is a particular clinical and ethical challenge because of the extremely poor outcome. Transplantation in the ICU setting often presents insurmountable difficulties.
authors.affiliation: 2nd Pulmonary Department, Attikon University Hospital, Athens Medical School, National and Kapodistrian University of Athens, 1 Rimini Street, 12462, Haidari, Greece. papiris@otenet.gr
authors.forename: Spyros A
authors.initials: SA
authors.lastname: Papiris
authors.forename: Effrosyni D
authors.initials: ED
authors.lastname: Manali
authors.forename: Likurgos
authors.initials: L
authors.lastname: Kolilekas
authors.forename: Konstantinos
authors.initials: K
authors.lastname: Kagouridis
authors.forename: Christina
authors.initials: C
authors.lastname: Triantafillidou
authors.forename: Iraklis
authors.initials: I
authors.lastname: Tsangaris
authors.forename: Charis
authors.initials: C
authors.lastname: Roussos
mesh_terms: D000208:Acute Disease; D055371:Acute Lung Injury / Q000209:etiology / Q000503:physiopathology / Q000628:therapy; D000818:Animals; D006801:Humans; D054990:Idiopathic Pulmonary Fibrosis / Q000150:complications / Q000503:physiopathology* / Q000628:therapy*; D007362:Intensive Care Units; D012121:Respiration, Artificial / Q000379:methods; D012128:Respiratory Distress Syndrome / Q000209:etiology / Q000503:physiopathology / Q000628:therapy