{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,24]],"date-time":"2026-04-24T10:03:19Z","timestamp":1777024999623,"version":"3.51.4"},"reference-count":0,"publisher":"Georg Thieme Verlag KG","issue":"02","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Pneumologie"],"published-print":{"date-parts":[[2018,2]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Idiopathic pulmonary fibrosis (IPF) is a severe and often fatal disease with a median survival of 2\u200a\u2013\u200a4 years after diagnosis. Since the publication of the German IPF guideline in 2013 new treatment trials have been published, necessitating an update of the pharmacological therapy of IPF. Different from the previous guideline, the GRADE system was discarded and replaced by the Oxford evidence classification system which allows a more differentiated judgement. The following pharmacological therapies were rated not suitable for the treatment of IPF patients (recommendation A; evidence 1-b): triple therapy with prednisolone, azathioprine and acetyl-cysteine; imatinib; ambrisentan; bosentan; macitentan. A less clear but still negative recommendation (B, 1-b) was attributed to the treatment of IPF with the phosphodiesterase-5-inhibitor sildenafil and acetyl-cysteine monotherapy. In contrast to the international guideline antacid therapy as a general treatment for IPF was rated negative, based on conflicting results of recent analyses (recommendation C; evidence 4). An unanimous positive recommendation was granted for the antifibrotic drugs nintedanib and pirfenidone for the treatment of IPF (A, 1-a). For some open questions in the management of IPF patients for which firm evidence is lacking the guideline also offers recommendations based on expert consensus.<\/jats:p>","DOI":"10.1055\/s-0043-123035","type":"journal-article","created":{"date-parts":[[2018,1,16]],"date-time":"2018-01-16T23:39:55Z","timestamp":1516145995000},"page":"155-168","source":"Crossref","is-referenced-by-count":39,"title":["German Guideline for Idiopathic Pulmonary Fibrosis \u2013 Update on Pharmacological Therapies 2017"],"prefix":"10.1055","volume":"72","author":[{"given":"J\u00fcrgen","family":"Behr","sequence":"additional","affiliation":[{"name":"Medizinische Klinik und Poliklinik V, Klinikum der Universit\u00e4t M\u00fcnchen (LMU) und Asklepios Fachkliniken M\u00fcnchen-Gauting, Comprehensive Pneumology Center, Mitglied des Deutschen Zentrums f\u00fcr Lungenforschung"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Andreas","family":"G\u00fcnther","sequence":"additional","affiliation":[{"name":"Schwerpunkt Fibrosierende Lungenerkrankungen, Universit\u00e4tsklinikum Gie\u00dfen \u2013 Marburg, Standort Gie\u00dfen, Justus-Liebig-Universit\u00e4t Gie\u00dfen, sowie Agaplesion Pneumologische Klinik Waldhof-Elgershausen, University of Giessen Marburg Lung Center, Mitglied des Deutschen Zentrums f\u00fcr Lungenforschung"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Francesco","family":"Bonella","sequence":"additional","affiliation":[{"name":"Schwerpunkt interstitielle und seltene Lungenkrankheiten, Ruhrlandklinik, Universit\u00e4tsklinikum Essen"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Klaus","family":"Gei\u00dfler","sequence":"additional","affiliation":[{"name":"Patientenvertretung Lungenfibrose e.V., Essen"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Dirk","family":"Koschel","sequence":"additional","affiliation":[{"name":"Abteilung Innere Medizin\/Pneumologie, Fachkrankenhaus Coswig, Zentrum f\u00fcr Pneumologie, Allergologie, Beatmungsmedizin, Thoraxchirurgie"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Michael","family":"Kreuter","sequence":"additional","affiliation":[{"name":"Zentrum f\u00fcr interstitielle und seltene Lungenerkrankungen, Pneumologie und Beatmungsmedizin, Thoraxklinik, Universit\u00e4tsklinikum Heidelberg, Mitglied des Deutschen Zentrums f\u00fcr Lungenforschung"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Antje","family":"Prasse","sequence":"additional","affiliation":[{"name":"Klinik f\u00fcr Pneumologie, Medizinische Hochschule Hannover und Clinical Research Center Fraunhofer Institut ITEM, Mitglied des Deutschen Zentrums f\u00fcr Lungenforschung"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Nicolas","family":"Sch\u00f6nfeld","sequence":"additional","affiliation":[{"name":"Klinik f\u00fcr Pneumologie, Lungenklinik Heckeshorn, HELIOS Klinikum Emil von Behring, Berlin"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Helmut","family":"Sitter","sequence":"additional","affiliation":[{"name":"Institut f\u00fcr Theoretische Chirurgie, Philipps-Universit\u00e4t Marburg"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Joachim","family":"M\u00fcller-Quernheim","sequence":"additional","affiliation":[{"name":"Klinik f\u00fcr Pneumologie, Department Innere Medizin, Medizinische Fakult\u00e4t, Albert Ludwigs Universit\u00e4t, Freiburg"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ulrich","family":"Costabel","sequence":"additional","affiliation":[{"name":"Schwerpunkt interstitielle und seltene Lungenkrankheiten, Ruhrlandklinik, Universit\u00e4tsklinikum Essen"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"194","published-online":{"date-parts":[[2018,1,16]]},"container-title":["Pneumologie"],"original-title":[],"language":"de","link":[{"URL":"http:\/\/www.thieme-connect.de\/products\/ejournals\/pdf\/10.1055\/s-0043-123035.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2018,2,20]],"date-time":"2018-02-20T23:38:59Z","timestamp":1519169939000},"score":1,"resource":{"primary":{"URL":"http:\/\/www.thieme-connect.de\/DOI\/DOI?10.1055\/s-0043-123035"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2018,1,16]]},"references-count":0,"journal-issue":{"issue":"02","published-online":{"date-parts":[[2018,2,20]]},"published-print":{"date-parts":[[2018,2]]}},"URL":"https:\/\/doi.org\/10.1055\/s-0043-123035","relation":{},"ISSN":["0934-8387","1438-8790"],"issn-type":[{"value":"0934-8387","type":"print"},{"value":"1438-8790","type":"electronic"}],"subject":[],"published":{"date-parts":[[2018,1,16]]}}}