{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,9]],"date-time":"2026-10-09T11:08:50Z","timestamp":1791544130696,"version":"4.3.4"},"reference-count":34,"publisher":"Georg Thieme Verlag KG","issue":"04","license":[{"start":{"date-parts":[[2024,4,12]],"date-time":"2024-04-12T00:00:00Z","timestamp":1712880000000},"content-version":"vor","delay-in-days":11,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"funder":[{"name":"Roche Pharma AG"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Pneumologie"],"published-print":{"date-parts":[[2024,4]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>\n            Introduction Pirfenidone was the first anti-fibrotic drug approved in Europe in 2011 for the treatment of mild-to-moderate idiopathic pulmonary fibrosis.\n    <\/jats:p><jats:p>\n            Objectives To investigate the clinical course of mild-to-moderate idiopathic pulmonary fibrosis in pirfenidone-treated patients in a real-world setting.\n    <\/jats:p><jats:p>\n            Methods The non-interventional study was conducted at 18 sites in Germany from 6\/2014\u201312\/2016. Adult patients with mild-to-moderate idiopathic pulmonary fibrosis were treated with pirfenidone (escalated from 3\u00d71 to 3\u00d73 capsules of 267 mg\/day within 3 weeks) for 12 months. The observation period comprised 4 follow-up visits at months 3, 6, 9 and 12. Disease progression was defined as decrease of \u226510% in vital capacity or \u226515% in diffusing capacity of the lung for carbon monoxide (DLCO) and\/or \u226550m in 6-minute walking distance vs. baseline, or \u201clack of response\/progression\u201c as reason for therapy discontinuation.\n    <\/jats:p><jats:p>\n            Results A total of 51 patients (80.4% male, mean age 70.6 years) were included in the full analysis set. Disease progression at any visit was reported for 23 (67.6%) of 34 patients with available data. Over the course of the study, lung function parameters, physical resilience, impact of cough severity on quality of life, and the mean Gender, Age and Physiology Index (stage II) remained stable. In total, 29 patients (56.9%) experienced at least one adverse drug reaction (11 patients discontinued due to adverse drug reactions); serious adverse reactions were reported in 12 patients (23.5%).\n    <\/jats:p><jats:p>\n            Conclusions The results of this study are in line with the established benefit-risk profile of pirfenidone. Therefore, pirfenidone can be considered a valuable treatment option to slow disease progression in mild-to-moderate idiopathic pulmonary fibrosis. NCT02622477\n    <\/jats:p>","DOI":"10.1055\/a-2267-2074","type":"journal-article","created":{"date-parts":[[2024,4,12]],"date-time":"2024-04-12T23:01:17Z","timestamp":1712962877000},"page":"236-243","source":"Crossref","is-referenced-by-count":5,"title":["Clinical course of mild-to-moderate idiopathic pulmonary fibrosis during therapy with pirfenidone: Results of the non-interventional study AERplus"],"prefix":"10.1055","volume":"78","clinical-trial-number":[{"clinical-trial-number":"nct02622477","registry":"10.18810\/clinical-trials-gov"}],"author":[{"given":"Jens","family":"Schreiber","sequence":"additional","affiliation":[{"name":"Pneumonology, Otto-von-Guericke-Universit\u00e4t Magdeburg, Magdeburg, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wolfgang","family":"Sch\u00fctte","sequence":"additional","affiliation":[{"name":"Klinik f\u00fcr Innere Medizin II, Krankenhaus Martha-Maria Halle-D\u00f6lau, Halle, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wolfgang","family":"Koerber","sequence":"additional","affiliation":[{"name":"Abt. Pneumologie\/Beatmungsmedizin und Schlaflabor, Evangelisches Krankenhaus G\u00f6ttingen-Weende gGmbH, Bovenden-Lenglern, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bernd","family":"Seese","sequence":"additional","affiliation":[{"name":"Abt. Pneumologie, Thoraxzentrum Bezirk Unterfranken, M\u00fcnnerstadt, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dirk","family":"Koschel","sequence":"additional","affiliation":[{"name":"Innere Medizin und Pneumologie, Fachkrankenhaus Coswig, Coswig, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kathrin","family":"Neuland","sequence":"additional","affiliation":[{"name":"Global Scientific Communications, Roche Pharma AG, Grenzach-Wyhlen, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christian","family":"Groh\u00e9","sequence":"additional","affiliation":[{"name":"Klinik f\u00fcr Pneumologie, Evangelische Lungenklinik Berlin, Berlin, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"194","published-online":{"date-parts":[[2024,4,12]]},"reference":[{"key":"ref1","doi-asserted-by":"publisher","first-page":"e18","DOI":"10.1164\/rccm.202202-0399ST","article-title":"Idiopathic Pulmonary Fibrosis (an Update) and Progressive Pulmonary Fibrosis in Adults: An Official ATS\/ERS\/JRS\/ALAT Clinical Practice Guideline","volume":"205","author":"G Raghu","year":"2022","journal-title":"Am J Respir Crit Care Med"},{"key":"ref2","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.00957-2022","article-title":"Idiopathic pulmonary fibrosis: state of the art for 2023","volume":"61","author":"AJ Podolanczuk","year":"2023","journal-title":"Eur Respir J"},{"key":"ref3","doi-asserted-by":"publisher","first-page":"646","DOI":"10.1164\/ajrccm.161.2.ats3-00","article-title":"Idiopathic pulmonary fibrosis: diagnosis and treatment. International consensus statement. American Thoracic Society (ATS), and the European Respiratory Society (ERS)","volume":"161","author":"American Thoracic Society","year":"2000","journal-title":"Am J Respir Crit Care Med"},{"key":"ref4","doi-asserted-by":"publisher","first-page":"53","DOI":"10.1186\/1471-2466-13-53","article-title":"Idiopathic pulmonary fibrosis \u2013 a systematic review on methodology for the collection of epidemiological data","volume":"13","author":"J Kaunisto","year":"2013","journal-title":"BMC Pulm Med"},{"key":"ref5","doi-asserted-by":"publisher","first-page":"180077","DOI":"10.1183\/16000617.0077-2018","article-title":"The epidemiology of idiopathic pulmonary fibrosis and interstitial lung diseases at risk of a progressive-fibrosing phenotype","volume":"27","author":"AL Olson","year":"2018","journal-title":"Eur Respir Rev"},{"key":"ref6","doi-asserted-by":"publisher","first-page":"1602419","DOI":"10.1183\/13993003.02419-2016","article-title":"Prevalence and incidence of interstitial lung diseases in a multi-ethnic county of Greater Paris","volume":"50","author":"B Duchemann","year":"2017","journal-title":"Eur Respir J"},{"key":"ref7","doi-asserted-by":"publisher","first-page":"e44","DOI":"10.1164\/rccm.201807-1255ST","article-title":"Diagnosis of Idiopathic Pulmonary Fibrosis. An Official ATS\/ERS\/JRS\/ALAT Clinical Practice Guideline","volume":"198","author":"G Raghu","year":"2018","journal-title":"Am J Respir Crit Care Med"},{"key":"ref8","doi-asserted-by":"publisher","first-page":"238","DOI":"10.1159\/000512315","article-title":"S2K Guideline for Diagnosis of Idiopathic Pulmonary Fibrosis","volume":"100","author":"J Behr","year":"2021","journal-title":"Respiration"},{"key":"ref9","doi-asserted-by":"publisher","first-page":"155","DOI":"10.1055\/s-0043-123035","article-title":"German Guideline for Idiopathic Pulmonary Fibrosis - Update on Pharmacological Therapies 2017","volume":"72","author":"J Behr","year":"2018","journal-title":"Pneumologie"},{"key":"ref10","doi-asserted-by":"publisher","first-page":"49","DOI":"10.1016\/j.rmed.2017.07.062","article-title":"Therapeutic targets in idiopathic pulmonary fibrosis","volume":"131","author":"M Kolb","year":"2017","journal-title":"Respir Med"},{"key":"ref11","doi-asserted-by":"publisher","first-page":"1040","DOI":"10.1164\/rccm.200404-571OC","article-title":"Double-blind, placebo-controlled trial of pirfenidone in patients with idiopathic pulmonary fibrosis","volume":"171","author":"A Azuma","year":"2005","journal-title":"Am J Respir Crit Care Med"},{"key":"ref12","doi-asserted-by":"publisher","first-page":"1760","DOI":"10.1016\/s0140-6736(11)60405-4","article-title":"Pirfenidone in patients with idiopathic pulmonary fibrosis (CAPACITY): two randomised trials","volume":"377","author":"PW Noble","year":"2011","journal-title":"Lancet"},{"key":"ref13","doi-asserted-by":"publisher","first-page":"821","DOI":"10.1183\/09031936.00005209","article-title":"Pirfenidone in idiopathic pulmonary fibrosis","volume":"35","author":"H Taniguchi","year":"2010","journal-title":"Eur Respir J"},{"key":"ref14","doi-asserted-by":"publisher","first-page":"2083","DOI":"10.1056\/NEJMoa1402582","article-title":"A phase 3 trial of pirfenidone in patients with idiopathic pulmonary fibrosis","volume":"370","author":"TE King Jr","year":"2014","journal-title":"N Engl J Med"},{"key":"ref15","doi-asserted-by":"publisher","first-page":"94","DOI":"10.1055\/a-1983-6796","article-title":"Pharmacological treatment of idiopathic pulmonary fibrosis (update) and progressive pulmonary fibrosis - S2k Guideline of the German Respiratory Society","volume":"77","author":"J Behr","year":"2023","journal-title":"Pneumologie"},{"key":"ref16","doi-asserted-by":"publisher","first-page":"e3","DOI":"10.1164\/rccm.201506-1063ST","article-title":"An Official ATS\/ERS\/JRS\/ALAT Clinical Practice Guideline: Treatment of Idiopathic\n          Pulmonary Fibrosis. An Update of the 2011 Clinical Practice Guideline","volume":"192","author":"G Raghu","year":"2015","journal-title":"Am J Respir Crit Care Med"},{"key":"ref17","doi-asserted-by":"publisher","first-page":"408","DOI":"10.1159\/000479976","article-title":"An Open-Label Study of the Long-Term Safety of Pirfenidone in Patients with Idiopathic Pulmonary Fibrosis (RECAP)","volume":"94","author":"U Costabel","year":"2017","journal-title":"Respiration"},{"key":"ref18","doi-asserted-by":"publisher","first-page":"00084-2018","DOI":"10.1183\/23120541.00084-2018","article-title":"Long-term safety of pirfenidone: results of the prospective, observational PASSPORT study","volume":"4","author":"V Cottin","year":"2018","journal-title":"ERJ Open Res"},{"key":"ref19","doi-asserted-by":"publisher","first-page":"684","DOI":"10.7326\/0003-4819-156-10-201205150-00004","article-title":"A multidimensional index and staging system for idiopathic pulmonary fibrosis","volume":"156","author":"B Ley","year":"2012","journal-title":"Ann Intern Med"},{"key":"ref20","doi-asserted-by":"publisher","first-page":"339","DOI":"10.1136\/thorax.58.4.339","article-title":"Development of a symptom specific health status measure for patients with chronic cough: Leicester Cough Questionnaire (LCQ)","volume":"58","author":"SS Birring","year":"2003","journal-title":"Thorax"},{"key":"ref21","doi-asserted-by":"publisher","first-page":"619","DOI":"10.1378\/chest.113.3.619","article-title":"Validation of a new dyspnea measure: the UCSD Shortness of Breath Questionnaire. University of California, San Diego","volume":"113","author":"EG Eakin","year":"1998","journal-title":"Chest"},{"key":"ref22","doi-asserted-by":"publisher","first-page":"199","DOI":"10.1159\/000363064","article-title":"Pirfenidone in idiopathic pulmonary fibrosis: real-life experience from a German tertiary referral center for interstitial lung diseases","volume":"88","author":"U Oltmanns","year":"2014","journal-title":"Respiration"},{"key":"ref23","doi-asserted-by":"publisher","first-page":"518","DOI":"10.1055\/s-0032-1332930","article-title":"Clinical experience with pirfenidone for the treatment of idiopathic pulmonary fibrosis","volume":"138","author":"F Bonella","year":"2013","journal-title":"Dtsch Med Wochenschr"},{"key":"ref24","doi-asserted-by":"publisher","first-page":"58","DOI":"10.1183\/09059180.00011514","article-title":"Long-term clinical and real-world experience with pirfenidone in the treatment of idiopathic pulmonary fibrosis","volume":"24","author":"V Cottin","year":"2015","journal-title":"Eur Respir Rev"},{"key":"ref25","doi-asserted-by":"publisher","first-page":"1395","DOI":"10.1513\/AnnalsATS.201606-458OC","article-title":"Acute Exacerbation and Decline in Forced Vital Capacity Are Associated with Increased Mortality in Idiopathic Pulmonary Fibrosis","volume":"14","author":"MO Paterniti","year":"2017","journal-title":"Ann Am Thorac Soc"},{"key":"ref26","doi-asserted-by":"publisher","first-page":"711","DOI":"10.1164\/rccm.201508-1546OC","article-title":"Predictors of Mortality Poorly Predict Common Measures of Disease Progression in Idiopathic Pulmonary Fibrosis","volume":"194","author":"B Ley","year":"2016","journal-title":"Am J Respir Crit Care Med"},{"key":"ref27","doi-asserted-by":"publisher","first-page":"73","DOI":"10.1159\/000504763","article-title":"Idiopathic Pulmonary Fibrosis: Best Practice in Monitoring and Managing a Relentless Fibrotic Disease","volume":"99","author":"WA Wuyts","year":"2020","journal-title":"Respiration"},{"key":"ref28","doi-asserted-by":"publisher","first-page":"1162","DOI":"10.1164\/rccm.201612-2492OC","article-title":"Effect of Emphysema Extent on Serial Lung Function in Patients with Idiopathic Pulmonary Fibrosis","volume":"196","author":"V Cottin","year":"2017","journal-title":"Am J Respir Crit Care Med"},{"key":"ref29","doi-asserted-by":"publisher","first-page":"186","DOI":"10.1183\/09031936.00217614","article-title":"Management of patients with idiopathic pulmonary fibrosis in clinical practice: the INSIGHTS-IPF registry","volume":"46","author":"J Behr","year":"2015","journal-title":"Eur Respir J"},{"key":"ref30","doi-asserted-by":"publisher","first-page":"3229","DOI":"10.3390\/biomedicines10123229","article-title":"Long-Term Safety of Antifibrotic Drugs in IPF: A Real-World Experience","volume":"10","author":"S Levra","year":"2022","journal-title":"Biomedicines"},{"key":"ref31","doi-asserted-by":"publisher","first-page":"1431","DOI":"10.1016\/j.rmed.2013.06.011","article-title":"Safety and efficacy of pirfenidone in idiopathic pulmonary fibrosis in clinical practice","volume":"107","author":"R Okuda","year":"2013","journal-title":"Respir Med"},{"key":"ref32","doi-asserted-by":"publisher","first-page":"177","DOI":"10.1186\/s12890-018-0736-z","article-title":"Pirfenidone improves survival in IPF: results from a real-life study","volume":"18","author":"GA Margaritopoulos","year":"2018","journal-title":"BMC Pulm Med"},{"key":"ref34","doi-asserted-by":"publisher","first-page":"72","DOI":"10.1016\/j.rmed.2019.06.001","article-title":"Patient journey and treatment patterns in adults with IPF based on health care data in Sweden from 2001 to 2015","volume":"155","author":"CM Sk\u00f6ld","year":"2019","journal-title":"Respir Med"},{"key":"ref35","doi-asserted-by":"publisher","first-page":"853","DOI":"10.1177\/08971900211008625","article-title":"Adherence, Persistence, and Effectiveness in Real Life. Multicenter Long-Term Study on the Use of Pirfenidone and Nintedanib in the Treatment of Idiopathic Pulmonary Fibrosis","volume":"35","author":"F Santoleri","year":"2022","journal-title":"J Pharm Pract"}],"container-title":["Pneumologie"],"original-title":[],"language":"de","link":[{"URL":"http:\/\/www.thieme-connect.de\/products\/ejournals\/pdf\/10.1055\/a-2267-2074.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,11,5]],"date-time":"2024-11-05T12:41:57Z","timestamp":1730810517000},"score":1,"resource":{"primary":{"URL":"http:\/\/www.thieme-connect.de\/DOI\/DOI?10.1055\/a-2267-2074"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,4]]},"references-count":34,"journal-issue":{"issue":"04","published-online":{"date-parts":[[2024,4,12]]},"published-print":{"date-parts":[[2024,4]]}},"URL":"https:\/\/doi.org\/10.1055\/a-2267-2074","archive":["Portico","CLOCKSS"],"relation":{},"ISSN":["0934-8387","1438-8790"],"issn-type":[{"value":"0934-8387","type":"print"},{"value":"1438-8790","type":"electronic"}],"subject":[],"published":{"date-parts":[[2024,4]]}}}