Survival and course of lung function in the presence or absence of antifibrotic treatment in patients with idiopathic pulmonary fibrosis: long-term results of the INSIGHTS-IPF registry

被引：119

作者：

Behr, Juergen ^{[1
,2
,3
,4
,5
]}

Prasse, Antje ^{[5
,6
,7
]}

Wirtz, Hubert ^{[8
]}

Koschel, Dirk ^{[9
]}

Pittrow, David ^{[10
,11
]}

Held, Matthias ^{[12
]}

Klotsche, Jens ^{[13
]}

Andreas, Stefan ^{[14
,15
]}

Claussen, Martin ^{[5
,16
]}

Grohe, Christian ^{[17
]}

Wilkens, Henrike ^{[18
]}

Hagmeyer, Lars ^{[19
,20
]}

Skowasch, Dirk ^{[21
]}

Meyer, Joachim F. ^{[22
]}

Kirschner, Joachim ^{[23
]}

Glaeser, Sven ^{[24
,25
]}

Kahn, Nicolas ^{[26
]}

Welte, Tobias ^{[5
,6
]}

Neurohr, Claus ^{[27
]}

Schwaiblmair, Martin ^{[28
]}

Bahmer, Thomas ^{[16
,29
]}

Oqueka, Tim ^{[30
]}

Frankenberger, Marion ^{[2
,3
]}

Kreuter, Michael ^{[5
,26
]}

机构：

[1] Univ Munich, LMU Klinikum, Med Klin & Poliklin 5, March Str 15, D-81377 Munich, Germany

[2] LMU Klinikum, Comprehens Pneumol Ctr CPC, Lungenforsch Ambulanz, Munich, Germany

[3] Helmholtz Zentrum, Munich, Germany

[4] Asklepios Fachkliniken, Munich, Germany

[5] German Ctr Lung Res DZL, Munich, Germany

[6] Hannover Med Sch, Klin Pneumol, Hannover, Germany

[7] Fraunhofer Inst ITEM, Hannover, Germany

[8] Univ Klinikum Leipzig AoR, Dept Innere Med Neurol & Dermatol, Abt Pneumol, Leipzig, Germany

[9] Fachkrankenhaus Coswig, Zentrum Pneumol, Coswig, Germany

[10] Tech Univ Dresden, Inst Klin Pharmakol, Med Fak, Dresden, Germany

[11] GWT TUD GmbH, Pharmacoepidemiol, Dresden, Germany

[12] Med Mission Hosp, Dept Internal Med Resp Med & Ventilatory Support, Cent Clin, Wurzburg, Germany

[13] Deutsch Rheuma Forschungszentrum, Epidemiol, Berlin, Germany

[14] Lungenfachklin Immenhausen, Gottingen, Germany

[15] Univ Med Gottingen, Kardiol & Pneumol, Gottingen, Germany

[16] LungenClin Grosshansdorf, Grosshansdorf, Germany

[17] Berlin Buch, Klin Pneumol ELK, Berlin, Germany

[18] Univ Kliniken Saarlandes, Univ Klinikum, Pneumol, Klin Innere Med 5, Homburg, Germany

[19] Univ Cologne, Zentrum Schlaf & Beatmungsmed, Krankenhaus Bethanien, Klin Pneumol & Allergol, Solingen, Germany

[20] Univ Cologne, Inst Pneumol, Solingen, Germany

[21] Univ Klinikum, Med Klin & Poliklin 2, Bonn, Germany

[22] Stadt Klinikum Munchen GmbH, LZM Bogenhausen Harlaching, Lungenzentrum Munchen, Munich, Germany

[23] Ctr Internal Med Studies CIMS, Bamberg, Germany

[24] Univ Med Greifswald, Forsch Bereich Pneumol & Pneumol Epidemiol, Klin & Poliklin Innere Med B, Greifswald, Germany

[25] Vivantes Klinikum Neukolln & Spandau, Klin Innere Med Pneumol & Infektiol, Berlin, Germany

[26] Heidelberg Univ, Ctr Interstitial & Rare Lung Dis, Thoraxklin, Pneumol, Heidelberg, Germany

[27] Klin Schillerhohe, Abt Pneumol & Beatmungsmed, Gerlingen, Germany

[28] Univ Klinikum, Med Klin, Augsburg, Germany

[29] Univ Klinikum Schleswig Holstein, Abt Innere Med 1, Campus Kiel, Kiel, Germany

[30] Univ Klinikum, Med Klin & Poliklin 22, Hamburg, Germany

来源：

EUROPEAN RESPIRATORY JOURNAL | 2020年 / 56卷 / 02期

关键词：

CLINICAL-PRACTICE; DOUBLE-BLIND; PIRFENIDONE; ACETYLCYSTEINE; MANAGEMENT; THERAPY; SAFETY;

D O I：

10.1183/13993003.02279-2019

中图分类号：

R56 [呼吸系及胸部疾病];

学科分类号：

摘要：

Objective: There is a paucity of observational data on antifibrotic therapy for idiopathic pulmonary fibrosis (IPF). We aimed to assess the course of disease of IPF patients with and without antifibrotic therapy under real-life conditions. Methods: We analysed data from a non-interventional, prospective cohort study of consecutively enrolled IPF patients from 20 interstitial lung disease expert centres in Germany. Data quality was ensured by automated plausibility checks, on-site monitoring, and source data verification. Propensity scores were applied to account for known differences in baseline characteristics between patients with and without antifibrotic therapy. Results: Among the 588 patients suitable for analysis, the mean +/- so age was 69.8 +/- 9.1 years, and 81.0% were male. The mean +/- so duration of disease since diagnosis was 1.8 +/- 3.4 years. The mean +/- SD value at baseline for forced vital capacity (FVC) and diffusion capacity (D-LCO) were 68.6 +/- 18.8% predicted and 37.8 +/- 18.5% predicted, respectively. During a mean +/- so follow-up of 1.2 +/- 0.7 years, 194 (33.0%) patients died. The 1-year and 2-year survival rates were 87% versus 46% and 62% versus 21%, respectively, for patients with versus without antifibrotic therapy. The risk of death was 37% lower in patients with antifibrotic therapy (hazard ratio 0.63, 95% CI 0.45; 0.87; p=0.005). The results were robust (and remained statistically significant) on multivariable analysis. Overall decline of FVC and D-LCO was slow and did not differ significantly between patients with or without antifibrotic therapy. Conclusions: Survival was significantly higher in IPF patients with antifibrotic therapy, but the course of lung function parameters was similar in patients with and without antifibrotic therapy. This su vests that in clinical practice, premature mortality of IPF patients eventually occurs despite stable measurements for FVC and D-LCO.

引用

页数：10

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