• Jürgen Behr, Michael Kreuter, Marius M Hoeper, Hubert Wirtz, Jens Klotsche, Dirk Koschel, Stefan Andreas, Martin Claussen, Christian Grohé, Henrike Wilkens, Winfried Randerath, Dirk Skowasch, F Joachim Meyer, Joachim Kirschner, Sven Gläser, Felix J F Herth, Tobias Welte, Rudolf Maria Huber, Claus Neurohr, Martin Schwaiblmair, Martin Kohlhäufl, Gert Höffken, Matthias Held, Andrea Koch, Thomas Bahmer, and David Pittrow.
• Study steering committee members j.behr@asklepios.com.
• Eur. Respir. J. 2015 Jul 1; 46 (1): 186-96.

AbstractAfter 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.Copyright ©ERS 2015.

30 keywords...

hide…