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. 2021 Dec 17:12:790204.
doi: 10.3389/fphar.2021.790204. eCollection 2021.

Outcomes and Incidence of PF-ILD According to Different Definitions in a Real-World Setting

Sebastiano Emanuele Torrisi  1   2 , Nicolas Kahn  1 , Julia Wälscher  1 , Markus Polke  1 , Joyce S Lee  3 , Philip L Molyneaux  4   5 , Francesca Maria Sambataro  6 , Claus Peter Heussel  6 , Carlo Vancheri  2 , Michael Kreuter  1
Affiliations

Outcomes and Incidence of PF-ILD According to Different Definitions in a Real-World Setting

Sebastiano Emanuele Torrisi et al. Front Pharmacol. .

Abstract

Background: Almost one-third of fibrosing ILD (fILDs) have a clinical disease behavior similar to IPF, demonstrating a progressive phenotype (PF-ILD). However, there are no globally accepted criteria on the definition of a progressive phenotype in non-IPF fILD yet. Four different definitions have been used; however, no internationally accepted definition currently exists. Research Question: To compare the clinical and functional characteristics of progressive fILD according to the currently available definitions. Study design and methods: Cases of fILD were identified retrospectively from the database of the tertiary referral center for ILD in Heidelberg. Lung function, clinical signs of progression, and radiological changes were evaluated. Patients with fILD were considered to have progression according to each of the four available definitions: Cottin (CO), RELIEF (RE), INBUILD (IN), and UILD study. Lung function changes, expressed as mean absolute decline of FVC%, were reported every 3 months following diagnosis and analyzed in the context of each definition. Survival was also analyzed. Results: A total of 566 patients with non-IPF fILD were included in the analysis. Applying CO-, RE-, IN-, and UILD-definitions, 232 (41%), 183 (32%), 274 (48%), and 174 (31%) patients were defined as PF-ILD, respectively. RE- and UILD-criteria were the most stringent, with only 32 and 31% patients defined as progressive, while IN- was the most broad, with almost 50% of patients defined as progressive. CO- definition was in-between, classifying 41% as progressive. PF ILD patients with a UILD definition had worse prognosis. Interpretation: Depending on the definition used, the existing criteria identify different groups of patients with progressive fILD, and this may have important prognostic and therapeutic implications.

Keywords: CTD-ILD; connective tissue disease; PF-ILD; fibrosing interstitial lung disease; non specific interstitial pneumonia; unclassifiable idiopathic interstitial pneumonia.

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Conflict of interest statement

The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.

Figures

FIGURE 1
FIGURE 1
Prevalence of PF-ILD according to COTTIN, RELIEF, INBUILD, and UILD study definitions.
FIGURE 2
FIGURE 2
PF-ILD definitions.
FIGURE 3
FIGURE 3
(A) FVC change of non PF versus PF-ILD according to COTTIN definition; (B) FVC change of non PF versus PF-ILD according to RELIEF definition; (C) FVC change of non PF versus PF-ILD according to INBUILD definition; (D) FVC change of non PF versus PF-ILD according to UILD study definition.
FIGURE 4
FIGURE 4
Comparison of FVC decline according to all four PF-ILD definition.
FIGURE 5
FIGURE 5
Frequency of PF-ILD definitions.
FIGURE 6
FIGURE 6
Survival of PF-ILD versus non PF-ILD compared to IPF, according to each definitions.
FIGURE 7
FIGURE 7
Comparison of survival of PF-ILD definitions with IPF.
See this image and copyright information in PMC

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