title: [Idiopathic pulmonary fibrosis: modern guideline-concordant diagnostics and innovative treatment].
abstract: Interstitial lung diseases are a heterogenic group of diseases who share in                     common manifestation of the disease process in the distal airways and                     alevolo-capilary membrane. Idiopathic pulmonary fibrosis (IPF) is of special                     interest as it is the most prevalent entity within the idiopathic interstitial                     pneumonias and it is associated with the worst prognosis. Diagnosis of IPF                     necessitates confirmation of a "usual interstitial pneumonia" (UIP) pattern on                     high-resolution computed tomography or on histology from a surgical lung biopsy.                     The recently published evidence-based IPF-guidelines for the first time define                     radiologic criteria for a UIP pattern and thus allow to make an IPF diagnosis in                     the absence of a surgical lung biopsy. Moreover, an IPF diagnosis can be made                     only after exclusion of any other cause for an interstitial lung disease.In the new IPF-guideline no medical therapy has received a positive                     recommendation. Based on the available evidence only pirfenidone has been shown                     to attenuate disease progression in mild to moderate IPF populations, with                     low-moderate quality of evidence. Pirfenidone has been approved for                     mild-moderate IPF in the European Union in February 2011 and is approved in                     Japan since 2009. In IPF-patients who cannot receive pirfenidone high-dose                     N-acetylcysteine is an "off-label-use" option that also has been shown to slow                     disease progression, however, based on low quality of evidence only. Recent                     studies suggest that anti-inflammatory therapies (prednione, azathioprine) may                     be harmful in IPF patients and cannot be recommended. Besides medical therapies                     IPF patients should receive best supportive care, which includes long-term                     oxygen therapy in hypoxemic patients and pulmonary rehabilitation as well as                     lung transplantation for eligible patients.
authors.affiliation: Medizinischen Klinik III, Berufsgenossenschaftliches Universitätsklinikum Bergmannsheil GmbH der Ruhr-Universität Bochum. juergen.behr@bergmansheil.de
authors.forename: J
authors.initials: J
authors.lastname: Behr
mesh_terms: D000111:Acetylcysteine / Q000627:therapeutic use; D000465:Algorithms; D000894:Anti-Inflammatory Agents, Non-Steroidal / Q000627:therapeutic use; D000925:Anticoagulants / Q000627:therapeutic use; D000975:Antioxidants / Q000627:therapeutic use; D001706:Biopsy; D005100:Expectorants / Q000627:therapeutic use; D006801:Humans; D054990:Idiopathic Pulmonary Fibrosis / Q000175:diagnosis* / Q000534:rehabilitation / Q000628:therapy*; D008168:Lung / Q000473:pathology; D016040:Lung Transplantation; D010102:Oxygen Inhalation Therapy; D017410:Practice Guidelines as Topic; D011728:Pyridones / Q000627:therapeutic use; D014057:Tomography, X-Ray Computed
chemical_list: D000894:Anti-Inflammatory Agents, Non-Steroidal; D000925:Anticoagulants; D000975:Antioxidants; D005100:Expectorants; D011728:Pyridones; C093844:pirfenidone; D000111:Acetylcysteine