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. 2018 Oct 9;5(1):e000342.
doi: 10.1136/bmjresp-2018-000342. eCollection 2018.

Acute exacerbation of idiopathic pulmonary fibrosis: a 10-year single-centre retrospective study

Masatoshi Yamazoe  1 , Hiromi Tomioka  1
Affiliations

Acute exacerbation of idiopathic pulmonary fibrosis: a 10-year single-centre retrospective study

Masatoshi Yamazoe et al. BMJ Open Respir Res. .

Abstract

Introduction: In 2016, an international working group proposed a revised definition and new diagnostic criteria for the acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF). Based on these criteria, AE-IPF was diagnosed regardless of the presence or absence of a known trigger and categorised as triggered (T-AE) or idiopathic (I-AE) AE-IPF. However, the clinical characteristics of the newly defined AE-IPF and clinical differences between T-AE and I-AE are unresolved.

Methods: We retrospectively analysed 64 patients with AE-IPF (I-AE (42), T-AE (22)) admitted to our hospital over a 10- year period.

Results: I-AE and T-AE cases did not show differences in in-hospital and long-term outcomes (in-hospital mortality: I-AE 52.4%, T-AE 59.1%, p=0.61; long-term mortality: p=0.68). In the I-AE group, significantly more patients received corticosteroid therapy before an AE (I-AE 35.7%, T-AE 4.5%; p=0.01). Significantly more patients in the T-AE group had lung cancer (I-AE 7.1%, T-AE 59.1%, p<0.001). I-AE occurred more frequently in winter while T-AE did not show seasonality. The white blood cell (WBC) count and haemoglobin (Hb) level were independent predictors of in-hospital deaths in I-AE (WBC: OR 1.87; 95% CI 1.09 to 4.95, p=0.01; Hb: OR 0.26, 95% CI 0.04 to 0.78, p=0.01) but not T-AE.

Discussion: With the introduction of new criteria for AE-IPF, a retrospective study over a 10-year period showed a lack of prognostic difference between I-AE and T-AE. The WBC count and Hb level predicted in-hospital outcome in I-AE cases.

Keywords: acute exacerbation; idiopathic pulmonary fibrosis; outcome; predictor; seasonality; triggered.

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Conflict of interest statement

Competing interests: None declared.

Figures

Figure 1
Figure 1
Case selection chart. AE-IPF, acute exacerbation of idiopathic pulmonary fibrosis; HOT, home oxygen therapy; IPF, idiopathic pulmonary fibrosis.
Figure 2
Figure 2
Seasonal variation in the occurrence of AE-IPF. AE-IPF, acute exacerbation of idiopathic pulmonary fibrosis.
Figure 3
Figure 3
Survival after hospitalisation, idiopathic AE versus triggered AE. AE, acute exacerbation.
See this image and copyright information in PMC

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