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Respir Res. 2019 Mar 15;20(1):59. doi: 10.1186/s12931-019-1020-3.

The clinical course of idiopathic pulmonary fibrosis and its association to quality of life over time: longitudinal data from the INSIGHTS-IPF registry.

Author information

1
Center for interstitial and rare lung diseases, Thoraxklinik, University of Heidelberg, Röntgenstr 1, D-69126, Heidelberg, Germany. kreuter@uni-heidelberg.de.
2
German Center for Lung Research, gießen, Germany. kreuter@uni-heidelberg.de.
3
Interstitial Lung Disease Program, National Jewish Health, Denver, CO, USA.
4
Institut für Klinische Pharmakologie, Medizinische Fakultät, Technische Universität Dresden, Dresden, Germany.
5
Department Market Access, Boehringer Ingelheim, Ingelheim am Rhein, Germany.
6
Epidemiologie, Deutsches Rheuma-Forschungsinstitut, Berlin, Germany.
7
Klinik für Pneumologie, Medizinische Hochschule Hannover, Hannover, Germany.
8
Fraunhofer Institute ITEM, Hannover, Germany.
9
German Center for Lung Research, gießen, Germany.
10
Abteilung für Pneumologie, Department Innere Medizin, Neurologie und Dermatologie, Universitätsklinikum Leipzig AöR, Leipzig, Germany.
11
Zentrum für Pneumologie und Bereich Pneumologie, Fachkrankenhaus Coswig und Uniklinikum Dresden, Coswig, Germany.
12
Lungenfachklinik Immenhausen and Universitätsmedizin Göttingen, Kardiologie und Pneumologie, Göttingen, Germany.
13
Lungen Clinic Grosshansdorf, Grosshansdorf, Germany.
14
Klinik für Pneumologie - ELK, Berlin Buch, Berlin, Germany.
15
Klinik für Innere Medizin V, Pneumologie, Universitätsklinikum Universitätskliniken des Saarlandes, Homburg, Germany.
16
Krankenhaus Bethanien, Solingen, Germany.
17
Medizinische Klinik und Poliklinik II, Universitätsklinikum Bonn, Bonn, Germany.
18
Lungenzentrum München, LZM Bogenhausen-Harlaching, Städtisches Klinikum München GmbH, Munich, Germany.
19
Center for Internal Medical Studies CIMS, Bamberg, Germany.
20
Klinik und Poliklinik für Innere Medizin B, Forschungsbereich Pneumologie und Pneumologische Epidemiologie, Universitätsmedizin Greifswald, Greifswald, Germany.
21
Vivantes Klinikum Spandau, Klinik für Innere Medizin, Berlin, Germany.
22
Center for interstitial and rare lung diseases, Thoraxklinik, University of Heidelberg, Röntgenstr 1, D-69126, Heidelberg, Germany.
23
Medizinische Klinik und Poliklinik V, Klinikum der LMU, Munich, Germany.
24
Schillerhöhe, Robert Bosch Krankenhaus, Stuttgart, Germany.
25
Medizinische Klinik, Klinikum Augsburg, Augsburg, Germany.
26
Klinikum Würzburg Mitte, Standort Missioklinik, Abteilung Innere Medizin, Pneumologie, Würzburg, Germany.
27
Universitätsklinikum Hamburg, Hamburg, Germany.
28
Asklepios Fachkliniken München-Gauting, Munich, Germany.

Abstract

BACKGROUND:

Quality of life (QoL) is profoundly impaired in patients with idiopathic pulmonary fibrosis (IPF). However, data is limited regarding the course of QoL. We therefore analysed longitudinal data from the German INSIGHTS-IPF registry.

METHODS:

Clinical status and QoL were assessed at enrollment and subsequently at 6- to 12-months intervals. A range of different QoL questionnaires including the St. George's Respiratory Questionnaire (SGRQ) were used.

RESULTS:

Data from 424 patients were included; 76.9% male; mean age 68.7 ± 9.1 years, mean FVC% predicted 75.9 ± 19.4, mean DLCO% predicted 36.1 ± 15.9. QoL worsened significantly during follow-up with higher total SGRQ scores (increased by 1.47 per year; 95% CI: 1.17 to 1.76; p < 0.001) and higher UCSD-SOBQ scores and lower EQ-5D VAS and WHO-5 scores. An absolute decline in FVC% predicted of > 10% was associated with a significant deterioration in SGRQ (increasing by 9.08 units; 95% CI: 2.48 to 15.67; p = 0.007), while patients with stable or improved FVC had no significantly change in SGRQ. Patients with a > 10% decrease of DLCO % predicted also had a significant increase in SGRQ (+ 7.79 units; 95% CI: 0.85 to 14.73; p = 0.028), while SQRQ was almost stable in patients with stable or improved DLCO. Patients who died had a significant greater increase in SGRQ total scores (mean 11.8 ± 18.6) at their last follow-up visit prior to death compared to survivors (mean 4.2 ± 18.9; HR = 1.03; 95% CI: 1.01 to 1.04; p < 0.001). All QoL scores across the follow-up period were significantly worse in hospitalised patients compared to non-hospitalised patients, with the worst scores reported in those hospitalised for acute exacerbations.

CONCLUSIONS:

QoL assessments in the INSIGHTS-IPF registry demonstrate a close relationship between QoL and clinically meaningful changes in lung function, comorbidities, disease duration and clinical course of IPF, including hospitalisation and mortality.

KEYWORDS:

Cohort study; Idiopathic pulmonary fibrosis; Patient-related outcomes; SQRQ

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