Asymmetry in acute exacerbation of idiopathic pulmonary fibrosis

Akihiko Sokai; Kiminobu Tanizawa; Tomohiro Handa; Takeshi Kubo; Seishu Hashimoto; Kohei Ikezoe; Yoshinari Nakatsuka; Kensaku Aihara; Yoshio Taguchi; Shigeo Muro; Toru Oga; Sonoko Nagai; Takateru Izumi; Toyohiro Hirai; Kazuo Chin; Michiaki Mishima

doi:10.1183/23120541.00036-2016

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FIGURE 1
Evaluation by high-resolution computed tomography (HRCT). The extents of emphysema, ground-glass opacities (GGOs), consolidation, and reticulation and honeycombing (R/HC) were scored to the nearest 10% in each of the five HRCT slices. The scores of 10 zones were averaged to obtain the mean scores for each lung. The right-to-left ratio of the parenchymal opacities consisting of GGOs and consolidation was calculated. This case, in which the ratio was 0.42, was an example of asymmetrical acute exacerbation.
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FIGURE 2
A comparison of 180-day survival curves between patients with idiopathic pulmonary fibrosis with asymmetrical acute exacerbation (AE) and those with symmetrical AE.

TABLE 1

Patient characteristics and clinical data

Characteristic
Subjects n	59
Males	54 (91.5)
Age years	71.7±8.2
Smoking history	49 (86.0)
FVC before AE % predicted	71.0±21.9
DL_CO before AE % predicted	37.0±17.3
Treatment before AE
LTOT	23 (39.0)
Corticosteroids	20 (33.9)
Immunosuppressive agents	10 (16.9)
WBC µL⁻¹	10 490±4150
D-dimer µg·mL⁻¹	5.1±6.3
CRP mg·dL⁻¹	6.5±6.4
LDH IU·L⁻¹	380±226
KL-6 U·mL⁻¹	1763±1278
P/F<200	26 (47.3)
BAL performed	11 (18.6)
Treatment of AE
High-dose corticosteroids	59 (100)
Antibiotics	58 (98.3)
Immunosuppressive agents	30 (50.8)
Noninvasive ventilation	14 (23.7)
180-day mortality %	59.2

The data are presented as mean±sd or n (%), unless otherwise indicated. FVC: forced vital capacity; AE: acute exacerbation; DL_CO: diffusing capacity of the lung for carbon monoxide; LTOT: long-term oxygen therapy; WBC: white blood cell; CRP: C-reactive protein; LDH: lactate dehydrogenase; KL-6: Krebs von den Lungen-6; P/F: the ratio of partial pressure of arterial oxygen to the fraction of inspired oxygen; BAL: bronchoalveolar lavage.

TABLE 2

Chest high-resolution computed tomography indices at diagnosis

Indices	Extent or number
Parenchymal opacities %	40.5±15.1
Ground-glass opacities %	34.1±15.1
Consolidation %	6.4±7.6
Reticulation/honeycombing %	14.8±12.1
Emphysema %	7.0±10.2
Bronchiectasis in parenchymal opacities	28 (47.5)
Asymmetrical acute exacerbation	13 (22.0)

The data are presented as mean±sd or n (%).

TABLE 3

Univariate and multivariate analyses of 180-day mortality

	Univariate analysis			Multivariate analysis
	HR	95% CI	p-value	HR	95% CI	p-value
Age	1.00	0.95–1.05	0.96
Male	1.97	0.59–12.2	0.30
Smoking history	0.44	0.20–1.11	0.08	0.51	0.23–1.31	0.15
LTOT	2.10	1.06–4.25	0.03*	2.34	1.04–5.28	0.04*
FVC before AE % predicted	0.99	0.97–1.01	0.43
DL_CO before AE % predicted	0.98	0.94–1.01	0.16
P/F <200	0.69	0.33–1.40	0.30
CRP	1.03	0.96–1.95	0.36
LDH 10 IU·L⁻¹ per unit	1.02	1.01–1.03	<0.01*	1.02	1.00–1.05	0.02*
KL-6 100 U·mL⁻¹ per unit	1.02	1.00–1.04	0.10	0.99	0.96–1.02	0.69
Parenchymal opacities	1.02	1.00–1.04	0.09	0.99	0.96–1.02	0.51
Ground-glass opacities	1.01	0.99–1.03	0.27
Consolidation	1.02	0.98–1.06	0.26
Reticulation/honeycombing	0.98	0.95–1.02	0.31
Emphysema	1.02	0.98–1.05	0.34
Bronchiectasis in parenchymal opacities	1.19	0.60–2.38	0.62
Asymmetrical AE	0.33	0.10–0.85	0.02*	0.36	0.10–0.99	0.047*

HR: hazard ratio; LTOT: long-term oxygen therapy; FVC: forced vital capacity; AE: acute exacerbation; DL_CO: diffusing capacity of the lung for carbon monoxide; P/F: the ratio of partial pressure of arterial oxygen to the fraction of inspired oxygen; CRP: C-reactive protein; LDH: lactate dehydrogenase; KL-6: Krebs von den Lungen-6. *: p<0.05.

TABLE 4

Patient characteristics between asymmetrical and symmetrical acute exacerbation

	Asymmetrical AE	Symmetrical AE	p-value
Subjects n	13	46
Age years	74.2±8.1	71.0±8.1	0.21
Male	10 (76.9)	44 (95.7)	0.07
Smoking history	3 (60.0)	21 (91.3)	0.14
FVC before AE % predicted	70.2±17.6	71.3±23.3	0.70
DL_CO before AE % predicted	34.6±20.5	38.0±16.2	0.32
Treatment before AE
LTOT	4 (30.8)	19 (41.3)	0.54
Corticosteroids	2 (15.4)	18 (39.1)	0.18
Immunosuppressive agents	0 (0.0)	10 (21.7)	0.10
WBC ×10³·µL⁻¹	10 120±4720	10 590±4020	0.43
D-dimer µg·mL⁻¹	4.7±5.3	5.3±6.7	1.00
CRP mg·dL⁻¹	5.7±4.6	6.7±6.8	0.96
LDH IU·L⁻¹	347±88	389±252	0.82
KL-6 U·mL⁻¹	1607±1074	1805±1335	0.54
P/F<200	8 (61.5)	18 (42.9)	0.34
BAL performed	1 (7.7)	10 (21.7)	0.43
Parenchymal opacities %	32.6±14.5	42.7±14.6	0.04*
Ground-glass opacities %	24.1±14.4	37.0±14.2	<0.01*
Consolidation %	8.6±9.2	5.7±7.0	0.18
Reticulation/honeycombing %	16.5±11.3	14.3±12.4	0.41
Emphysema %	11.7±13.1	5.7±9.0	0.07
Bronchiectasis in parenchymal opacities	6 (47.5)	22 (47.8)	1.00
Treatment of AE
High-dose corticosteroids	13 (100)	46 (100)	–
Antibiotics	13 (100)	45 (97.8)	1.00
Immunosuppressive agents	4 (30.8)	26 (56.5)	0.13
Non-invasive ventilation	3 (23.1)	11 (23.9)	1.00
180-day mortality %	30.8	68.2	0.03*

The data are presented as mean±sd or n (%), unless otherwise indicated. AE: acute exacerbation; FVC: forced vital capacity; DL_CO: diffusing capacity of the lung for carbon monoxide; LTOT: long-term oxygen therapy; WBC: white blood cell; CRP: C-reactive protein; LDH: lactate dehydrogenase; KL-6: Krebs von den Lungen-6; P/F: the ratio of partial pressure of arterial oxygen to the fraction of inspired oxygen; BAL: bronchoalveolar lavage. *: p<0.05.