Shun Kondoh1, Hirofumi Chiba2, Hirotaka Nishikiori3, Yasuaki Umeda4, Koji Kuronuma5, Mitsuo Otsuka6, Gen Yamada7, Hirofumi Ohnishi8, Mitsuru Mori9, Yasuhiro Kondoh10, Hiroyuki Taniguchi11, Sakae Homma12, Hiroki Takahashi13. 1. Department of Respiratory Medicine and Allergology, Sapporo Medical University School of Medicine, South-1 West-16 Chuo-ku, Sapporo, Hokkaido 060-8556, Japan. Electronic address: shun_kondo@sapmed.ac.jp. 2. Department of Respiratory Medicine and Allergology, Sapporo Medical University School of Medicine, South-1 West-16 Chuo-ku, Sapporo, Hokkaido 060-8556, Japan. Electronic address: hchiba@sapmed.ac.jp. 3. Department of Respiratory Medicine and Allergology, Sapporo Medical University School of Medicine, South-1 West-16 Chuo-ku, Sapporo, Hokkaido 060-8556, Japan. Electronic address: hnishiki@sapmed.ac.jp. 4. Department of Respiratory Medicine and Allergology, Sapporo Medical University School of Medicine, South-1 West-16 Chuo-ku, Sapporo, Hokkaido 060-8556, Japan. Electronic address: y.umeda@sapmed.ac.jp. 5. Department of Respiratory Medicine and Allergology, Sapporo Medical University School of Medicine, South-1 West-16 Chuo-ku, Sapporo, Hokkaido 060-8556, Japan. Electronic address: kuronumak@sapmed.ac.jp. 6. Department of Respiratory Medicine and Allergology, Sapporo Medical University School of Medicine, South-1 West-16 Chuo-ku, Sapporo, Hokkaido 060-8556, Japan. Electronic address: ohtsukam@sapmed.ac.jp. 7. Department of Respiratory Medicine and Allergology, Sapporo Medical University School of Medicine, South-1 West-16 Chuo-ku, Sapporo, Hokkaido 060-8556, Japan. Electronic address: gyamada@sapmed.ac.jp. 8. Department of Public Health, Sapporo Medical University School of Medicine, Japan. Electronic address: hohnishi@sapmed.ac.jp. 9. Department of Public Health, Sapporo Medical University School of Medicine, Japan. Electronic address: mitsurum@sapmed.ac.jp. 10. Department of Respiratory Medicine and Allergy, Tosei General Hospital, Japan. Electronic address: konyasu2005@yahoo.co.jp. 11. Department of Respiratory Medicine and Allergy, Tosei General Hospital, Japan. Electronic address: taniguchi@tosei.or.jp. 12. Division of Respiratory Medicine, Toho University Omori Medical Center, Japan. Electronic address: sahomma@med.toho-u.ac.jp. 13. Department of Respiratory Medicine and Allergology, Sapporo Medical University School of Medicine, South-1 West-16 Chuo-ku, Sapporo, Hokkaido 060-8556, Japan. Electronic address: htaka@sapmed.ac.jp.
Abstract
BACKGROUND: The clinical course of idiopathic pulmonary fibrosis (IPF) shows great inter-individual differences. It is important to standardize the severity classification to accurately evaluate each patient׳s prognosis. In Japan, an original severity classification (the Japanese disease severity classification, JSC) is used. In the United States, the new multidimensional index and staging system (the GAP model) has been proposed. The objective of this study was to evaluate the model performance for the prediction of mortality risk of the JSC and GAP models using a large cohort of Japanese patients with IPF. METHODS: This is a retrospective cohort study including 326 patients with IPF in the Hokkaido prefecture from 2003 to 2007. We obtained the survival curves of each stage of the GAP and JSC models to perform a comparison. In the GAP model, the prognostic value for mortality risk of Japanese patients was also evaluated. RESULTS: In the JSC, patient prognoses were roughly divided into two groups, mild cases (Stages I and II) and severe cases (Stages III and IV). In the GAP model, there was no significant difference in survival between Stages II and III, and the mortality rates in the patients classified into the GAP Stages I and II were underestimated. CONCLUSIONS: It is difficult to predict accurate prognosis of IPF using the JSC and the GAP models. A re-examination of the variables from the two models is required, as well as an evaluation of the prognostic value to revise the severity classification for Japanese patients with IPF.
BACKGROUND: The clinical course of idiopathic pulmonary fibrosis (IPF) shows great inter-individual differences. It is important to standardize the severity classification to accurately evaluate each patient׳s prognosis. In Japan, an original severity classification (the Japanese disease severity classification, JSC) is used. In the United States, the new multidimensional index and staging system (the GAP model) has been proposed. The objective of this study was to evaluate the model performance for the prediction of mortality risk of the JSC and GAP models using a large cohort of Japanese patients with IPF. METHODS: This is a retrospective cohort study including 326 patients with IPF in the Hokkaido prefecture from 2003 to 2007. We obtained the survival curves of each stage of the GAP and JSC models to perform a comparison. In the GAP model, the prognostic value for mortality risk of Japanese patients was also evaluated. RESULTS: In the JSC, patient prognoses were roughly divided into two groups, mild cases (Stages I and II) and severe cases (Stages III and IV). In the GAP model, there was no significant difference in survival between Stages II and III, and the mortality rates in the patients classified into the GAP Stages I and II were underestimated. CONCLUSIONS: It is difficult to predict accurate prognosis of IPF using the JSC and the GAP models. A re-examination of the variables from the two models is required, as well as an evaluation of the prognostic value to revise the severity classification for Japanese patients with IPF.
Authors: Rebeccah M Brusca; Iago Pinal-Fernandez; Kevin Psoter; Julie J Paik; Jemima Albayda; Christopher Mecoli; Eleni Tiniakou; Andrew L Mammen; Lisa Christopher-Stine; Sonye Danoff; Cheilonda Johnson Journal: Respir Med Date: 2019-02-21 Impact factor: 3.415
Authors: Tejaswini Kulkarni; Kaiyu Yuan; Thi K Tran-Nguyen; Young-Il Kim; Joao A de Andrade; Tracy Luckhardt; Vincent G Valentine; Daniel J Kass; Steven R Duncan Journal: PLoS One Date: 2019-10-04 Impact factor: 3.240
Authors: Toby M Maher; Elisabeth Bendstrup; Louis Dron; Jonathan Langley; Gerald Smith; Javaria Mona Khalid; Haridarshan Patel; Michael Kreuter Journal: Respir Res Date: 2021-07-07