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 (SLB) 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 (NAC) 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 (RCTs). In addition to HRCT, the frequency of lung biopsies was surprisingly high. Treatment patterns varied substantially.
Abstract
This largest published registry of IPF patients shows surprising disease severity and treatment variation http://ow.ly/JbWRn
Footnotes
This article has supplementary material available from erj.ersjournals.com
Clinical trial: This study is registered at www.clinicaltrials.gov with identifier number NCT01695408.
Support statement: Boehringer Ingelheim AG & Co. KG, Germany, funded the study with an unrestricted educational grant. Boehringer Ingelheim did not influence the study design, analysis or interpretation of the study. The open access for this article was paid for by Boehringer Ingelheim. Funding information for this article has been deposited with FundRef.
Conflict of interest: Disclosures can be found alongside the online version of this article at erj.ersjournals.com
- Received May 7, 2014.
- Accepted February 3, 2015.
- Copyright ©ERS 2015
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