title: Management of patients with idiopathic pulmonary fibrosis in clinical practice: the INSIGHTS-IPF registry.
abstract: After introduction of the new international guidelines on idiopathic pulmonary fibrosis (IPF) in 2011, we investigated clinical management practices for patients with IPF according to physicians' diagnoses. A prospective, multicenter, noninterventional study with comprehensive quality measures including on-site source data verification was performed in Germany. 502 consecutive patients (171 newly diagnosed, 331 prevalent; mean±SD age 68.7±9.4 years, 77.9% males) with a mean disease duration of 2.3±3.5 years were enrolled. IPF diagnosis was based on clinical assessments and high-resolution computed tomography (HRCT) in 90.2%, and on surgical lung biopsy combined with histology in 34.1% (lavage in 61.8%). The median 6-min walk distance was 320 m (mean 268±200 m). The mean forced vital capacity was 72±20% pred and diffusing capacity of the lung for carbon monoxide was 35±15% pred. No drugs were administered in 17.9%, oral steroids in 23.7%, N-acetylcysteine in 33.7%, pirfenidone in 44.2% and other drugs in 4.6% of patients. Only 2.8% of the cohort was listed for lung transplantation. IPF patients were diagnosed in line with the new guidelines. They had more severe disease than those enrolled in recent randomised controlled trials. In addition to HRCT, the frequency of lung biopsies was surprisingly high. Treatment patterns varied substantially.
authors.affiliation: Study steering committee members j.behr@asklepios.com.
authors.forename: Jürgen
authors.initials: J
authors.lastname: Behr
authors.affiliation: Study steering committee members.
authors.forename: Michael
authors.initials: M
authors.lastname: Kreuter
authors.affiliation: Study steering committee members.
authors.forename: Marius M
authors.initials: MM
authors.lastname: Hoeper
authors.affiliation: Study steering committee members.
authors.forename: Hubert
authors.initials: H
authors.lastname: Wirtz
authors.affiliation: Study steering committee members.
authors.forename: Jens
authors.initials: J
authors.lastname: Klotsche
authors.affiliation: For the authors' affiliations, see the Acknowledgements Study steering committee members.
authors.forename: Dirk
authors.initials: D
authors.lastname: Koschel
authors.affiliation: For the authors' affiliations, see the Acknowledgements Study steering committee members.
authors.forename: Stefan
authors.initials: S
authors.lastname: Andreas
authors.affiliation: For the authors' affiliations, see the Acknowledgements Study steering committee members.
authors.forename: Martin
authors.initials: M
authors.lastname: Claussen
authors.affiliation: For the authors' affiliations, see the Acknowledgements Study steering committee members.
authors.forename: Christian
authors.initials: C
authors.lastname: Grohé
authors.affiliation: For the authors' affiliations, see the Acknowledgements Study steering committee members.
authors.forename: Henrike
authors.initials: H
authors.lastname: Wilkens
authors.affiliation: For the authors' affiliations, see the Acknowledgements Study steering committee members.
authors.forename: Winfried
authors.initials: W
authors.lastname: Randerath
authors.affiliation: For the authors' affiliations, see the Acknowledgements Study steering committee members.
authors.forename: Dirk
authors.initials: D
authors.lastname: Skowasch
authors.affiliation: For the authors' affiliations, see the Acknowledgements Study steering committee members.
authors.forename: F Joachim
authors.initials: FJ
authors.lastname: Meyer
authors.affiliation: For the authors' affiliations, see the Acknowledgements Study steering committee members.
authors.forename: Joachim
authors.initials: J
authors.lastname: Kirschner
authors.affiliation: For the authors' affiliations, see the Acknowledgements Study steering committee members.
authors.forename: Sven
authors.initials: S
authors.lastname: Gläser
authors.affiliation: For the authors' affiliations, see the Acknowledgements Study steering committee members.
authors.forename: Felix J F
authors.initials: FJ
authors.lastname: Herth
authors.affiliation: For the authors' affiliations, see the Acknowledgements Study steering committee members.
authors.forename: Tobias
authors.initials: T
authors.lastname: Welte
authors.affiliation: For the authors' affiliations, see the Acknowledgements Study steering committee members.
authors.forename: Rudolf Maria
authors.initials: RM
authors.lastname: Huber
authors.affiliation: For the authors' affiliations, see the Acknowledgements Study steering committee members.
authors.forename: Claus
authors.initials: C
authors.lastname: Neurohr
authors.affiliation: For the authors' affiliations, see the Acknowledgements Study steering committee members.
authors.forename: Martin
authors.initials: M
authors.lastname: Schwaiblmair
authors.affiliation: For the authors' affiliations, see the Acknowledgements Study steering committee members.
authors.forename: Martin
authors.initials: M
authors.lastname: Kohlhäufl
authors.affiliation: For the authors' affiliations, see the Acknowledgements Study steering committee members.
authors.forename: Gert
authors.initials: G
authors.lastname: Höffken
authors.affiliation: For the authors' affiliations, see the Acknowledgements Study steering committee members.
authors.forename: Matthias
authors.initials: M
authors.lastname: Held
authors.affiliation: For the authors' affiliations, see the Acknowledgements Study steering committee members.
authors.forename: Andrea
authors.initials: A
authors.lastname: Koch
authors.affiliation: For the authors' affiliations, see the Acknowledgements Study steering committee members.
authors.forename: Thomas
authors.initials: T
authors.lastname: Bahmer
authors.affiliation: Study steering committee members.
authors.forename: David
authors.initials: D
authors.lastname: Pittrow
mesh_terms: D000111:Acetylcysteine / Q000627:therapeutic use; D000368:Aged; D000894:Anti-Inflammatory Agents, Non-Steroidal / Q000627:therapeutic use; D001706:Biopsy; D005080:Exercise Test; D005260:Female; D005858:Germany; D006801:Humans; D054990:Idiopathic Pulmonary Fibrosis / Q000000981:diagnostic imaging / Q000503:physiopathology* / Q000628:therapy*; D008168:Lung / Q000473:pathology / Q000502:physiology; D008297:Male; D008875:Middle Aged; D017410:Practice Guidelines as Topic; D011446:Prospective Studies; D011728:Pyridones / Q000627:therapeutic use; D012042:Registries; D013256:Steroids / Q000008:administration & dosage; D014057:Tomography, X-Ray Computed; D014797:Vital Capacity
chemical_list: D000894:Anti-Inflammatory Agents, Non-Steroidal; D011728:Pyridones; D013256:Steroids; C093844:pirfenidone; D000111:Acetylcysteine
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