Table 2 Multivariate fine and Gray competing risk regression model for the development of AE-IIP adjusted for age, gender, and %predicted FVC.

Variable	Hazard ratio	95% CI	p-value	q-value*
IPAF, +	0.423	0.098–1.821	0.248	0.400
UIP pattern on HRCT, +	0.467	0.187–1.165	0.103	0.269
Honeycomb lung with HRCT, +	0.985	0.450–2.153	0.969	0.969
UIP pattern on Pathology, +	0.918	0.255–3.305	0.895	0.969
ΔFVC in one year, % pred	0.965	0.942–0.988	0.003	0.027
DL_CO, % pred ≥ 68% (median), +	0.754	0.327–1.739	0.508	0.762
ΔDL_CO in one year, % pred.	0.970	0.937–1.005	0.090	0.269
Mucocutaneous lesion, +	1.147	0.350–3.753	0.821	0.969
Joint lesion, +	1.711	0.258–11.364	0.578	0.810
Dry symptoms or findings, +	0.953	0.229–3.973	0.947	0.969
LDH ≥ 220 U/L (median), +	1.713	0.836–3.508	0.141	0.330
KL-6, U/mL	1.000	1.000–1.000	0.072	0.269
SP-D, mg/dL	1.001	0.999–1.003	0.246	0.400
CRP, mg/dL	0.980	0.879–1.092	0.714	0.938
Anti-CCP antibody, +	4.407	1.608–12.075	0.004	0.027
RF ≥ 30†, +	0.312	0.044–2.231	0.246	0.400
PM/DM-, SSc-related antibodies** or ANA ≥ 320†, +	< 0.001‡	0.000 – <0.001	< 0.001	< 0.001
Steroid treatment only before AE-IIP, +	0.545	0.208–1.428	0.217	0.400
Immunosuppressive treatment before AE-IIP§, +	0.479	0.202–1.137	0.095	0.269
Anti-fibrotic treatment before AE-IIP, +	0.505	0.228–1.119	0.092	0.269
Development of systemic autoimmune diseases, +	1.068	0.153–7.447	0.947	0.969

* q-values represent the p-values adjusted for multiple comparisons using Storey’s q-value method, which controls the false discovery rate.
** PM/DM- or SSc-related antibodies include anti-ARS antibody, anti-Jo-1 antibody, anti-Scl70 antibody, anti-RNP antibody, anti-RNA polymerase III antibody, and anti-centromere antibody.
† Thresholds of RF ≥ 30 and ANA ≥ 320 were set based on the criteria for IPAF (Fischer, A. et al. Eur Respir J 46, 976–987 (2015).).
‡ Numerical instability for small number of event issue.
§ Immunosuppressive therapy was defined as corticosteroid and/or immunosuppressants.
Abbreviations; CI: confidence interval, IPF: idiopathic pulmonary fibrosis, FVC: forced vital capacity, DL_CO: diffusion lung capacity for carbon monoxide, HRCT: high-resolution computed tomography, UIP: usual interstitial pneumonia, LDH: lactate dehydrogenase, KL-6: Krebs von den Lungen-6, SP-D: surfactant protein-D, AE: acute exacerbation, ANA: antinuclear antibody, ANCA: anti-neutrophil cytoplasmic antibody, RF: rheumatoid factor, CCP: cyclic citrullinated peptide, ARS: aminoacyl tRNA synthetase, CRP: C reactive protein, IPAF: interstitial pneumonia with autoimmune features, PM/DM: polymyositis/dermatomyositis, SSc: systemic sclerosis.

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