Validation of the Japanese disease severity classification and the GAP model in Japanese patients with idiopathic pulmonary fibrosis

Kondoh, Shun; Chiba, Hirofumi; Nishikiori, Hirotaka; Umeda, Yasuaki; Kuronuma, Koji; Otsuka, Mitsuo; Yamada, Gen; Ohnishi, Hirofumi; Mori, Mitsuru; Kondoh, Yasuhiro; Taniguchi, Hiroyuki; Homma, Sakae; Takahashi, Hiroki

doi:10.1016/j.resinv.2016.02.009

Cited by 37 publications

(28 citation statements)

References 18 publications

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“…However, the existing IPF registries are relatively small in sample size and restricted to a single country, with only limited data from Central and Eastern Europe [12,18]. Moreover, the validation of valuable prognostic tools such as the GAP index in other populations has been constrained by the relatively small cohort sizes and short follow-up [19][20][21].…”

Section: Introductionmentioning

confidence: 99%

The European MultiPartner IPF registry (EMPIRE): validating long-term prognostic factors in idiopathic pulmonary fibrosis

Tran¹,

Šterclová²,

Moğulkoç³

et al. 2020

Respir Res

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Background: Several registries of idiopathic pulmonary fibrosis (IPF) have been established to better understand its natural history, though their size and duration of follow-up are limited. Here, we describe the large European MultiPartner IPF Registry (EMPIRE) and validate predictors of long-term survival in IPF. Methods: The multinational prospective EMPIRE registry enrolled IPF patients from 48 sites in 10 Central and Eastern European countries since 2014. Survival from IPF diagnosis until death was estimated, accounting for lefttruncation. The Cox proportional hazards regression model was used to estimate adjusted hazard ratios (HR) of death for prognostic factors, using restricted cubic splines to fit continuous factors. Results: The cohort included 1620 patients (mean age at diagnosis 67.6 years, 71% male, 63% smoking history), including 75% enrolled within 6 months of diagnosis. Median survival was 4.5 years, with 45% surviving 5 years post-diagnosis. Compared with GAP stage I, mortality was higher with GAP stages II (HR 2.9; 95% CI: 2.3-3.7) and III (HR 4.0; 95% CI: 2.8-5.7) while, with redefined cutoffs , the corresponding HRs were 2.7 (95% CI: 1.8-4.0) and 5.8 (95% CI: 4.0-8.3) respectively. Mortality was higher with concurrent pulmonary hypertension (HR 2.0; 95% CI: 1.5-2.9) and lung cancer (HR 2.6; 95% CI: 1.3-4.9). Conclusions: EMPIRE, one of the largest long-term registries of patients with IPF, provides a more accurate confirmation of prognostic factors and co-morbidities on longer term five-year mortality. It also suggests that some fine-tuning of the indices for mortality may provide a more accurate long-term prognostic profile for these patients.

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Section: Introductionmentioning

confidence: 99%

The European MultiPartner IPF registry (EMPIRE): validating long-term prognostic factors in idiopathic pulmonary fibrosis

Tran¹,

Šterclová²,

Moğulkoç³

et al. 2020

Respir Res

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“…Among a number of different methods, the gender, age, physiology (GAP) index and the GAP calculator for the GAP Risk Assessment System (GAP system) have proven to be the most easy and applicable tool in the current clinical practice; however, there are still only few studies that have assessed their applicability and usefulness in daily practice. Furthermore, ethnicity has been reported as a factor that can influence the reliability of these two scoring systems, as demonstrated by the Korean and Japanese experiences . Indeed, up until now most of the data have been derived from American studies .…”

Section: Introductionmentioning

confidence: 99%

“…Furthermore, ethnicity has been reported as a factor that can influence the reliability of these two scoring systems, as demonstrated by the Korean and Japanese experiences. 7,8 Indeed, up until now most of the data have been derived from American studies. 1 Finally, to our knowledge, there are still very few clinical trials that have evaluated the applicability of the GAP system in the era of antifibrotic therapies.…”

Section: Introductionmentioning

confidence: 99%

The prognostic role of Gender‐Age‐Physiology system in idiopathic pulmonary fibrosis patients treated with pirfenidone

Harari

Confalonieri²,

Poletti

et al. 2019

Clinical Respiratory J

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Introduction Gender, age, physiology (GAP) system have proven to be an easy tool for predicting disease stages and survival in idiopathic pulmonary fibrosis (IPF) patients. Objective To validate mortality risk as determined by the GAP system in a real‐life multicentre IPF population treated with pirfenidone. Methods The study included patients who received pirfenidone for at least 6 months. The GAP calculator and the GAP index were determined. The primary outcome was all‐cause mortality. The prognostic accuracy of the GAP system was evaluated with respect to calibration and discrimination. Results and Conclusion Sixty‐eight IPF patients were enrolled in the study. The median follow‐up was 2.4 years (range 0.1‐7.4 years). A total of 22 deaths as first event (32%) and of 10 lung transplantation (15%) were recorded. The cumulative incidence of mortality at 1, 2 and 3 years was 10.4%, 22.4% and 38.4%, respectively. The differences between the predicted and observed mortality were not significant for the GAP index while the observed mortality become comparable to that predicted by the GAP calculator only in the third year of follow‐up. The C‐index for the GAP index was 0.74 (95% CI 0.57‐0.93) while the C‐statistic value for the GAP calculator was 0.77 (95% CI 0.59‐0.95).

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“…There is no universal consensus on how to define disease severity of IPF patients, with several different methods including baseline FVC, diffusing capacity of lung for carbon monoxide (DL CO ), GAP (gender, age and physiology) index, composite physiological index (CPI) and Japanese disease severity classification (JSC) described. Whilst there is some difficulty using a single measure of FVC to judge severity of impairment in the clinical setting where a premorbid value is often unknown, it has been widely accepted as a measure of disease progression and despite its failings, has been repeatedly used as a primary outcome measure for clinical trials …”

Section: Pharmacological Therapymentioning

confidence: 99%

Treatment of idiopathic pulmonary fibrosis in Australia and New Zealand: A position statement from the Thoracic Society of Australia and New Zealand and the Lung Foundation Australia

Troy

Keir

et al. 2017

Respirology

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Idiopathic pulmonary fibrosis (IPF) is a fibrosing interstitial lung disease (ILD) of unknown aetiology with a median survival of only 2-5 years. It is characterized by progressive dyspnoea and worsening lung function, ultimately resulting in death. Until recently, there were no effective therapies for IPF; however, with the publication of two landmark clinical trials in 2014, the anti-fibrotic therapies, nintedanib and pirfenidone, have gained widespread approval. This position paper aims to highlight the current evidence for the treatment of IPF, with particular application to the Australian and New Zealand population. We also consider areas in which evidence is currently lacking, especially with regard to the broader IPF severity spectrum and treatment of co-morbid conditions. The utility of non-pharmacological therapies including pulmonary rehabilitation, oxygen as well as symptom management thought to be important in the holistic care of IPF patients are also discussed.

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Validation of the Japanese disease severity classification and the GAP model in Japanese patients with idiopathic pulmonary fibrosis

Cited by 37 publications

References 18 publications

The European MultiPartner IPF registry (EMPIRE): validating long-term prognostic factors in idiopathic pulmonary fibrosis

The European MultiPartner IPF registry (EMPIRE): validating long-term prognostic factors in idiopathic pulmonary fibrosis

The prognostic role of Gender‐Age‐Physiology system in idiopathic pulmonary fibrosis patients treated with pirfenidone

Treatment of idiopathic pulmonary fibrosis in Australia and New Zealand: A position statement from the Thoracic Society of Australia and New Zealand and the Lung Foundation Australia

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