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

Kreuter, Michael; Swigris, Jeff; Pittrow, David; Geier, Silke; Klotsche, Jens; Prasse, Antje; Wirtz, Hubert; Koschel, Dirk; Andreas, Stefan; Claussen, Martin; Grohé, Christian; Wilkens, Henrike; Hagmeyer, Lars; Skowasch, Dirk; Meyer, Joachim F.; Kirschner, Joachim; Gläser, Sven; Kahn, Nicolas; Welte, Tobias; Neurohr, Claus; Schwaiblmair, Martin; Held, Matthias; Bahmer, Thomas; Oqueka, Tim; Frankenberger, Marion; Behr, Jürgen

doi:10.1186/s12931-019-1020-3

Table 4 Multivariable association of QoL and clinical characteristics with any hospitalizations and hospitalizations due to exacerbations in follow-up

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

	SGRQ			EQ-5D VAS			UCSD-SOBQ			WHO-5
	beta	95% CI	p-value	beta	95% CI	p-value	beta	95% CI	p-value	beta	95% CI	p-value
Any hospitalisation	3.95	0.35; 7.56	0.032	− 0.53	−3.70; 2.64	0.743	8.41	2.46; 14.37	0.006	−0.56	− 1.60; 0.48	0.292
Age	−0.04	− 0.26; 0.17	0.702	− 0.21	− 0.39; − 0.02	0.026	0.26	− 0.08; 0.61	0.129	− 0.05	− 0.11; 0.02	0.141
Number of comorbidities
0	0.00			0.00			0.00			0.00
1	−1.61	−7.37; 4.15	0.584	−0.40	−5.40; 4.60	0.875	0.31	−8.64; 9.26	0.946	0.29	−1.25; 1.83	0.714
2	1.82	−3.91; 7.56	0.533	−3.01	−8.19; 2.17	0.255	5.47	−3.72; 14.66	0.243	0.20	−1.36; 1.76	0.801
3	3.36	−2.91; 9.62	0.293	−4.07	−9.51; 1.38	0.143	7.11	−2.70; 16.93	0.155	−0.96	−2.63; 0.71	0.259
4+	11.42	4.99; 17.85	0.001	−10.18	− 16.28; −4.07	0.001	22.96	11.27; 34.65	< 0.001	−2.64	−5.02; −0.27	0.029
Change in FVC % predicted
Stable/increase	0.00			0.00			0.00			0.00
Decrease by 0 to 10%	3.70	−0.10; 7.51	0.057	−2.93	−6.17; 0.32	0.077	4.60	−1.67; 10.87	0.151	−0.51	−1.62; 0.61	0.375
Decrease by > 10%	8.08	2.23; 13.93	0.007	−6.11	−11.14; − 1.08	0.017	8.91	−0.23; 18.05	0.056	−0.97	−2.59; 0.66	0.242
Overall physician’s judgement of clinical course of IPF
Stable disease	0.00			0.00			0.00			0.00
Slow progression	7.15	4.88; 9.42	< 0.001	−7.24	− 10.05; −4.44	< 0.001	10.65	5.96; 15.35	< 0.001	−1.62	−2.34; − 0.89	< 0.001
Rapid progression	22.68	16.83; 28.52	< 0.001	− 26.37	−32.73; − 20.01	< 0.001	33.57	24.66; 42.48	< 0.001	−6.35	−8.31; −4.40	< 0.001
No judgement possible	3.02	0.33; 5.70	0.027	−2.15	−5.16; 0.87	0.163	1.70	−2.96; 6.35	0.475	−1.25	−2.11; −0.40	0.004
Hospitalisation by exacerbation	5.35	0.64; 10.07	0.026	−1.56	−5.78; 2.65	0.468	10.89	2.72; 19.05	0.009	−0.78	−2.32; 0.76	0.320
Age	0.04	−0.24; 0.31	0.791	−0.37	− 0.59; − 0.15	0.001	0.40	− 0.02; 0.82	0.062	− 0.04	−0.12; 0.04	0.383
Number of comorbidities
0	0.00			0.00			0.00			0.00
1	−0.33	−6.99; 6.32	0.922	−1.35	−7.22; 4.51	0.651	1.41	−8.79; 11.61	0.786	0.23	−1.58; 2.04	0.804
2	2.93	−3.86; 9.72	0.397	−2.43	−8.49; 3.64	0.433	6.77	−4.36; 17.89	0.233	−0.09	−2.05; 1.88	0.930
3	3.52	−3.89; 10.93	0.352	−1.79	−8.17; 4.60	0.583	7.09	−5.08; 19.27	0.253	−0.94	−3.15; 1.26	0.403
4+	13.15	5.95; 20.36	< 0.001	−8.90	−15.99; − 1.82	0.014	21.07	8.29; 33.84	0.001	−2.60	−5.56; 0.36	0.085
Change in FVC % predicted
Stable/increase	0.00			0.00			0.00			0.00
Decrease by 0 to 10%	4.08	−0.73; 8.88	0.096	−4.12	−8.39; 0.15	0.059	6.63	−1.24; 14.50	0.099	−0.75	−2.17; 0.67	0.300
Decrease by > 10%	7.49	0.66; 14.32	0.032	−4.05	−10.00; 1.90	0.182	9.98	−0.91; 20.86	0.072	−1.04	−2.79; 0.72	0.247
Overall physician’s judgement of clinical course of IPF
Stable disease	0.00			0.00			0.00			0.00
Slow progression	6.65	3.84; 9.46	< 0.001	−8.90	−12.49; −5.31	< 0.001	9.98	3.38; 16.58	0.003	−1.63	−2.55; −0.70	0.001
Rapid progression	24.23	17.19; 31.26	< 0.001	−27.75	−36.15; −19.34	< 0.001	33.78	22.82; 44.75	< 0.001	−6.67	−9.06; −4.29	< 0.001
No judgement possible	2.36	−0.77; 5.49	0.139	−1.16	−4.97; 2.66	0.552	−0.76	−6.46; 4.94	0.794	−0.98	−2.00; 0.03	0.058

beta regression coefficient, CI confidence interval, EQ-5D VAS EuroQol five-dimensional questionnaire, recorded as a visual analog scale, FVC forced vital capacity, IPF idiopathic pulmonary fibrosis, QoL quality of life, SD standard deviation, SGRQ St. George’s Respiratory Questionnaire, USCD-SOBQ University of California San Diego Shortness of Breath Questionnaire, WHO-5 World Health Organization-5 Well-Being Index

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