Adjustment of GAP model for Japanese patients with idiopathic pulmonary fibrosis

Abstract Background Although antifibrotic drugs, including nintedanib and pirfenidone, slow the progression of idiopathic pulmonary fibrosis (IPF), there is little data about the timing of start of antifibrotic treatment in real-world clinical practice. The present study aimed to clarify the efficacy of nintedanib and pirfenidone in patients with early-stage IPF. Methods We compared survival and disease progression between patients with IPF with Japanese Respiratory Society (JRS) disease severity system stage I with and without oxygen desaturation on the 6-min walk test (6MWT) and increased the gender–age–physiology (GAP) staging. We examined the efficacy of antifibrotic drugs in patients with early-stage IPF. Results The severity of stage I IPF (n = 179) according to the JRS criteria consisted of the following GAP staging criteria: stage I, 111 cases; stage II, 58 cases; stage III, 10 cases. The duration from the initial visit to disease progression and survival time was significantly shorter in JRS stage I patients with oxygen desaturation on the 6MWT or with increased GAP staging (unfavorable group) compared with patients without those factors. In the unfavorable group, the relative decline in percentage predicted forced vital capacity (%FVC) over 6 months was significantly lower in patients undergoing antifibrotic treatment compared with non-treated patients. Conclusion Antifibrotic drugs have a beneficial effect on the decline in %FVC in Japanese patients with early-stage IPF who have oxygen desaturation on the 6MWT or increased GAP staging.

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Nintedanib in Japanese patients with idiopathic pulmonary fibrosis: A subgroup analysis of the INPULSIS® randomized trials

Respirology ◽

10.1111/resp.12960 ◽

2016 ◽

Vol 22 (4) ◽

pp. 750-757 ◽

Cited By ~ 19

Author(s):

Arata Azuma ◽

Hiroyuki Taniguchi ◽

Yoshikazu Inoue ◽

Yasuhiro Kondoh ◽

Takashi Ogura ◽

...

Keyword(s):

Idiopathic Pulmonary Fibrosis ◽

Pulmonary Fibrosis ◽

Subgroup Analysis ◽

Randomized Trials ◽

Japanese Patients

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Basis of Acute Exacerbation of Idiopathic Pulmonary Fibrosis in Japanese Patients

American Journal of Respiratory and Critical Care Medicine ◽

10.1164/ajrccm.177.12.1398 ◽

2008 ◽

Vol 177 (12) ◽

pp. 1398-1398 ◽

Cited By ~ 1

Author(s):

Harold R. Collard ◽

Fernando J. Martinez

Keyword(s):

Idiopathic Pulmonary Fibrosis ◽

Pulmonary Fibrosis ◽

Acute Exacerbation ◽

Japanese Patients

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A modified GAP model for East-Asian populations with idiopathic pulmonary fibrosis

Respiratory Investigation ◽

10.1016/j.resinv.2020.04.001 ◽

2020 ◽

Vol 58 (5) ◽

pp. 395-402

Author(s):

Hirotaka Nishikiori ◽

Hirofumi Chiba ◽

Sang Hoon Lee ◽

Shun Kondoh ◽

Ken-ichi Kamo ◽

...

Keyword(s):

Idiopathic Pulmonary Fibrosis ◽

Pulmonary Fibrosis ◽

East Asian ◽

Gap Model ◽

Asian Populations

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Baseline High-Resolution CT Findings Predict Acute Exacerbation of Idiopathic Pulmonary Fibrosis: German and Japanese Cohort Study

Journal of Clinical Medicine ◽

10.3390/jcm8122069 ◽

2019 ◽

Vol 8 (12) ◽

pp. 2069 ◽

Cited By ~ 4

Author(s):

Chihiro Hirano ◽

Shinichiro Ohshimo ◽

Yasushi Horimasu ◽

Hiroshi Iwamoto ◽

Kazunori Fujitaka ◽

...

Keyword(s):

High Resolution ◽

Idiopathic Pulmonary Fibrosis ◽

Pulmonary Fibrosis ◽

Acute Exacerbation ◽

Ground Glass Opacity ◽

Japanese Patients ◽

Computed Tomographic ◽

Japanese Cohort ◽

Kaplan Meier ◽

Lung Abnormalities

Acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) is a major cause of morbidity and death in IPF. However, sensitive predictive factors of AE-IPF have not been well-investigated. To investigate whether high-resolution computed tomographic (HRCT) abnormalities predict AE-IPF in independent ethnic cohorts, this study included 121 patients with IPF (54 German and 67 Japanese; mean age, 68.5 ± 7.6 years). Two radiologists independently visually assessed the presence and extent of lung abnormalities in each patient. Twenty-two (18.2%) patients experienced AE-IPF during the follow-up. The incidence of AE-IPF was significantly higher in the Japanese patients (n = 18, 26.9%) than in the German patients (n = 4, 7.3%, p < 0.01). In the Kaplan–Meier analysis, patients with a larger extent of ground glass opacity (GGO), fibrosis, and traction bronchiectasis experienced an earlier onset of AE-IPF (p = 0.0033, 0.0088, and 0.049, respectively). In the multivariate analysis, a larger extent of GGO and fibrosis on HRCT were independent predictors of AE-IPF (p = 0.026 and 0.037, respectively). Additionally, Japanese ethnicity was independently associated with the incidence of AE-IPF after adjustment for HRCT findings (p = 0.0074). In conclusion, a larger extent of GGO and fibrosis on HRCT and Japanese ethnicity appear to be risk factors for AE-IPF.

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Experience of Japanese Patients with Idiopathic Pulmonary Fibrosis who Cope with Dyspnea

Journal of Japan Academy of Nursing Science ◽

10.5630/jans.36.238 ◽

2016 ◽

Vol 36 (0) ◽

pp. 238-246 ◽

Cited By ~ 3

Author(s):

Yasuko Igai

Keyword(s):

Idiopathic Pulmonary Fibrosis ◽

Pulmonary Fibrosis ◽

Japanese Patients

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Unified baseline and longitudinal mortality prediction in idiopathic pulmonary fibrosis

European Respiratory Journal ◽

10.1183/09031936.00146314 ◽

2015 ◽

Vol 45 (5) ◽

pp. 1374-1381 ◽

Cited By ~ 42

Author(s):

Brett Ley ◽

Williamson Z. Bradford ◽

Derek Weycker ◽

Eric Vittinghoff ◽

Roland M. du Bois ◽

...

Keyword(s):

Idiopathic Pulmonary Fibrosis ◽

Pulmonary Fibrosis ◽

Risk Prediction ◽

Goodness Of Fit ◽

Model Performance ◽

Predictive Performance ◽

Gap Model ◽

Goodness Of Fit Test ◽

Net Reclassification Improvement ◽

C Statistic

The Gender-Age-Physiology (GAP) model is a validated, baseline-risk prediction model for mortality in idiopathic pulmonary fibrosis. Longitudinal variables have been shown to contribute to risk prediction in idiopathic pulmonary fibrosis and may improve the predictive performance of the baseline GAP model. Our aims were to further validate the GAP model and evaluate whether the addition of longitudinal variables improves its predictive performance.The study population was derived from a large clinical trials cohort of patients with idiopathic pulmonary fibrosis (n=1109). Model performance was determined by improvement in the C-statistic, net reclassification improvement, clinical net reclassification improvement, and a goodness-of-fit test.The GAP model had good discriminative performance with a C-statistic of 0.757 (95% CI 0.750–0.764). However, the original GAP model tended to overestimate risk in this cohort. A novel, easy to use model, consisting of the original GAP predictors plus history of respiratory hospitalisation and 24-week change in forced vital capacity (the longitudinal GAP model) improved model performance with a C-statistic of 0.785 (95% CI 0.780–0.790), net reclassification improvement of 8.5%, clinical net reclassification improvement of 25%, and a goodness-of-fit test of 0.929.The Longitudinal GAP model, along with the original GAP model, may unify baseline and longitudinal mortality risk prediction in idiopathic pulmonary fibrosis.

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