Estimates of epidemiology, mortality and disease burden associated with progressive fibrosing interstitial lung disease in France (the PROGRESS study)

Table 3 Final multivariable Cox model of factors associated with mortality

Parameter	Category	HR (95% CI)	p-value	Type 3 test p-value
Model 1. Factors associated with mortality in the first year post-progression (n = 14,413)
Sex	Female	1.00		< 0.0001
	Male	1.32 (1.23–1.42)	< 0.0001	< 0.0001
Categorised age^a	≥ 20–< 50	1.00		< 0.0001
	≥ 50–< 60	1.74 (1.44–2.09)	< 0.0001
	≥ 60–< 75	2.65 (2.27–3.10)	< 0.0001
	≥ 75–< 104	3.87 (3.33–4.51)	< 0.0001
Underlying disease^b	HP	1.00		< 0.0001
	Sarcoidosis-ILD	1.00 (0.80–1.25)	0.9916
	Other autoimmune^c	1.23 (0.99–1.52)	0.0569
	RA-ILD	1.53 (1.26–1.86)	< 0.0001
	Exposure-related ILD other than HP^d	1.57 (1.30–1.89)	< 0.0001
	MCTD-ILD	1.62 (1.29–2.04)	< 0.0001
	SSc-ILD	1.76 (1.42–2.20)	< 0.0001
	IIP	1.92 (1.59–2.32)	< 0.0001
Model 2. Factors associated with mortality after the first year post-progression (n = 8928)^e
Sex	Female	1.00		< 0.0001
	Male	1.32 (1.23–1.42)	< 0.0001	< 0.0001
Categorised age^a	≥ 20–< 50	1.00		< 0.0001
	≥ 50–< 60	1.31 (1.11–1.55)	0.0015
	≥ 60–< 75	2.58 (2.25–2.96)	< 0.0001
	≥ 75–< 104	4.43 (3.87–5.08)	< 0.0001
Underlying disease^b	HP	1.00		< 0.0001
	Sarcoidosis-ILD	0.94 (0.78–1.15)	0.5603
	Other autoimmune^c	0.97 (0.80–1.18)	0.7828
	RA-ILD	1.03 (0.86–1.23)	0.7455
	MCTD-ILD	1.08 (0.86–1.36)	0.4861
	IIP	1.13 (0.95–1.35)	0.1608
	Exposure-related ILD other than HP^d	1.32 (1.11–1.56)	0.0015
	SSc-ILD	1.64 (1.34–2.01)	< 0.0001

And for these models, the proportional hazards assumption was respected
CI confidence interval, HR hazard ratio, HP hypersensitivity pneumonitis, IIP idiopathic interstitial pneumonia, ILD interstitial lung disease, MCTD mixed connective tissue disease, PF-ILD progressive fibrosing interstitial lung disease, RA rheumatoid arthritis, SSc systemic sclerosis
^aAge categories were each compared with the 20–50 years age group
^bUnderlying diseases were each compared with the hypersensitivity pneumonitis subgroup
^cSjogren syndrome, polymyositis and systemic lupus erythematosus
^dCoal workers’ pneumoconiosis, asbestosis, pneumoconiosis due to other dust containing silica, aluminosis of the lung, bauxite fibrosis of the lung, berylliosis, graphite fibrosis of the lung, siderosis, pneumoconiosis due to other specified inorganic dusts, unspecified pneumoconiosis, byssinosis, cannabinosis, bronchitis and pneumonitis due to chemicals, gases, fumes and vapours, chronic respiratory conditions due to chemicals, gases, fumes and vapours, chronic and other pulmonary manifestations due to radiation, chronic drug-induced interstitial lung disorders, unspecified drug-induced interstitial lung disorders, respiratory conditions due to other specified external agents, respiratory conditions due to unspecified external agent
^ePatients who were still followed 1 year after progression

ISSN: 1465-993X