PT  - JOURNAL ARTICLE
AU  - Juergen Behr
AU  - Michael Kreuter
AU  - Marius Hoeper
AU  - Jens Klotsche
AU  - Hubert Wirtz
AU  - Dirk Koschel
AU  - Stefan Andreas
AU  - Martin Claussen
AU  - Christian Grohé
AU  - David Pittrow
TI  - Late-breaking abstract: Current management of patients with idiopathic pulmonary fibrosis in clinical practice: INSIGHTS-IPF registry
DP  - 2014 Sep 01
TA  - European Respiratory Journal
PG  - 1901
VI  - 44
IP  - Suppl 58
4099  - http://erj.ersjournals.com/content/44/Suppl_58/1901.short
4100  - http://erj.ersjournals.com/content/44/Suppl_58/1901.full
SO  - Eur Respir J2014 Sep 01; 44
AB  - In a prospective non-interventional registry, we are investigating current clinical management practices in Germany for consecutive patients with IPF according to physicians' diagnoses.On May 5th 2014, 405 patients were included (mean age 68.6 ± 9.6 years, 77.0% male). Onset of first symptoms was at 64.2 ± 11.1 years (4.1 ± 4.6 years before diagnosis), and the mean duration of disease since diagnosis was 2.4 ± 3.7 years. For IPF diagnosis high-resolution computed tomography (HRCT) was available in 89.9% and a surgical lung biopsy was obtained in 33.6%. Bronchoalveolar lavage was performed in 62.2% of the patients. The median six-minute walk distance was 322 meters (mean, 269 ± 201). The mean % of predicted forced vital capacity was 72 ± 20, and mean % predicted DLCO was 35± 15. No pharmacologic treatment of IPF was administered in 15.8% of the patients, oral steroids in 22.1% (as monotherapy in 7.1%); N-acetylcysteine in 34.8% (monotherapy in 11.9% and triple therapy with azathioprine and steroids in 1.5%), pirfenidone in 47.2% (monotherapy in 29.1%), other drugs in 5.4%, and long-term O2 therapy in 34.4%. Mean quality-of life on the 1-100 EQ-5D visual analogue scale was 54 ±26 points. In the physicians' overall judgement, 35.6% had stable disease, 31.1% slow progression, 11.9% rapid progression (21.5% no judgement possible). Only 3.2% of the cohort was listed for lung transplantation.The IPF patients in this large registry tended to have more severe disease than those enrolled in recent randomised controlled trials. In addition to HRCT, the frequency of surgical lung biopsies was surprisingly high. Treatment patterns varied substantially.