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01.12.2018 | Research article | Ausgabe 1/2018 Open Access

BMC Pulmonary Medicine 1/2018

Disease progression in idiopathic pulmonary fibrosis with mild physiological impairment: analysis from the Australian IPF registry

Zeitschrift:
BMC Pulmonary Medicine > Ausgabe 1/2018
Autoren:
Helen E. Jo, Ian Glaspole, Yuben Moodley, Sally Chapman, Samantha Ellis, Nicole Goh, Peter Hopkins, Greg Keir, Annabelle Mahar, Wendy Cooper, Paul Reynolds, E. Haydn Walters, Christopher Zappala, Christopher Grainge, Heather Allan, Sacha Macansh, Tamera J. Corte
Wichtige Hinweise

Electronic supplementary material

The online version of this article (https://​doi.​org/​10.​1186/​s12890-018-0575-y) contains supplementary material, which is available to authorized users.

Abstract

Background

Idiopathic pulmonary fibrosis (IPF) is a progressive and fatal fibrosing lung disease of unknown cause. The advent of anti-fibrotic medications known to slow disease progression has revolutionised IPF management in recent years. However, little is known about the natural history of IPF patients with mild physiological impairment. We aimed to assess the natural history of these patients using data from the Australian IPF Registry (AIPFR).

Methods

Using our cohort of real-world IPF patients, we compared FVC criteria for mild physiological impairment (FVC ≥ 80%) against other proposed criteria: DLco ≥ 55%; CPI ≤40 and GAP stage 1 with regards agreement in classification and relationship with disease outcomes. Within the mild cohort (FVC ≥ 80%), we also explored markers associated with poorer prognosis at 12 months.

Results

Of the 416 AIPFR patients (mean age 70.4 years, 70% male), 216 (52%) were classified as ‘mild’ using FVC ≥ 80%. There was only modest agreement between FVC and DLco (k = 0.30), with better agreement with GAP (k = 0.50) and CPI (k = 0.48). Patients who were mild had longer survival, regardless of how mild physiologic impairment was defined. There was, however, no difference in the annual decline in FVC% predicted between mild and moderate-severe groups (for all proposed criteria). For patients with mild impairment (n = 216, FVC ≥ 80%), the strongest predictor of outcomes at 12 months was oxygen desaturation on a 6 min walk test.

Conclusion

IPF patients with mild physiological impairment have better survival than patients with moderate-severe disease. Their overall rate of disease progression however, is comparable, suggesting that they are simply at different points in the natural history of IPF disease.
Zusatzmaterial
Additional file 1: Table S1. GAP stage calculation. (DOC 30 kb)
12890_2018_575_MOESM1_ESM.doc
Additional file 2: Table S2. Baseline characteristics of patients excluded from analysis. (DOC 35 kb)
12890_2018_575_MOESM2_ESM.doc
Additional file 3: Figure S1. Exploratory analysis of varying thresholds. (PNG 100 kb)
12890_2018_575_MOESM3_ESM.png
Additional file 4: Figure S2. Locally Weighted Scatterplot Smoothing curves for FVC% predicted. a. Mild physiological impairment (FVC ≥ 80%). b. Moderate-severe physiological impairment (FVC < 80%). c. Summary of LOWESS curve means. (ZIP 212 kb)
12890_2018_575_MOESM4_ESM.zip
Literatur
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