Observational Study

. 2025 May;63(3):365-372.

doi: 10.1016/j.resinv.2025.02.009. Epub 2025 Mar 17.

Prognostic prediction for newly diagnosed patients with idiopathic interstitial pneumonia: JIPS Registry (NEJ030)

Ryo Okuda¹, Takashi Ogura², Shu Hisata³, Tomohisa Baba¹, Yasuhiro Kondoh⁴, Takafumi Suda⁵, Takeshi Johkoh⁶, Tae Iwasawa⁷, Hiromi Tomioka⁸, Masashi Bando³, Arata Azuma⁹, Yoshikazu Inoue¹⁰, Nobuhisa Ishikawa¹¹, Naoki Arai¹², Takahisa Takihara¹³, Megumi Hamaguchi¹⁴, Toru Arai¹⁰, Yutaro Nakamura¹⁵, Atsushi Miyamoto¹⁶, Keisuke Tomii¹⁷, Yasunari Miyazaki¹⁸, Hirofumi Chiba¹⁹, Haruyuki Ishii²⁰, Naoki Hamada²¹, Yasuhiro Terasaki²², Junya Fukuoka²³, Fumikazu Sakai⁷, Ryoko Egashira²⁴, Kiminori Fujimoto²⁵, Hiromitsu Sumikawa²⁶, Takuji Suzuki²⁷, Susumu Sakamoto²⁸, Yasuhiko Nishioka²⁹, Noboru Hattori³⁰, Naozumi Hashimoto³¹, Satoshi Morita³², Nao Ichihara³³, Hiroaki Miyata³⁴, Koichi Hagiwara³, Kunihiko Kobayashi³⁵, Toshihiro Nukiwa³⁶; JIPS registry collaborators

Collaborators, Affiliations

Collaborators

JIPS registry collaborators:
Machiko Arita, Masahito Ebina, Yasutsugu Fukushima, Yoshihiro Funaki, Yasuhiro Gon, Tomohiro Handa, Hiromichi Hara, Dai Hashimoto, Seishu Hashimoto, Kazuya Ichikado, Hidenori Ichiyasu, Kazuyoshi Imaizumi, Minoru Inomata, Hiroshi Ishimoto, Hiroyuki Ito, Katsuyoshi Iwabuchi, Michiko Kagajo, Motoyasu Kato, Tomoya Katsuta, Tetsuji Kawamura, Masahiro Kimura, Yuichiro Kimura, Tomoo Kishaba, Yasuhiko Koga, Yasuo Kohashi, Chiyoko Kono, Kei Morikawa, Hiroaki Nakagawa, Osamu Narumoto, Hirotsugu Ohkubo, Masaki Okamoto, Yoshitaka Oyamada, Keita Sakamoto, Tomoya Sasaki, Shintaro Sato, Yusuke Shikama, Yasuo Shimizu, Shinko Suzuki, Kageaki Taima, Kazufumi Takamatsu, Motoaki Tanigawa, Yoshinori Tanino, Osamu Nishiyama, Mikio Toyoshima, Kosuke Tsuruno, Akifumi Uchida, Hirokazu Urushiyama, Kazuhiro Usui, Satoshi Wasamoto, Yuko Waseda, Satoshi Watanabe, Hiroshi Yamamoto, Kazuhiro Yatera, Koshi Yokomura

Affiliations

¹ Department of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory Center, 6-16-1 Tomioka-higashi, Kanazawa-ku, Yokohama, 236-0051, Japan.
² Department of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory Center, 6-16-1 Tomioka-higashi, Kanazawa-ku, Yokohama, 236-0051, Japan. Electronic address: takaoguogu@gmail.com.
³ Division of Pulmonary Medicine, Department of Medicine, Jichi Medical University, 3311-1 Yakushiji, Shimotsuke, 329-0498, Japan.
⁴ Department of Respiratory Medicine and Allergy, Tosei General Hospital, 160 Nishioiwake-cho, Seto, 489-8642, Japan.
⁵ Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, 1-20-1 Handayama, Tyuo-ku, Hamamatsu, 431-3192, Japan.
⁶ Department of Radiology, Kansai Rosai Hospital, 3-1-69 Inabaso, Amagasaki, 660-8511, Japan.
⁷ Department of Radiology, Kanagawa Cardiovascular and Respiratory Center, 6-16-1 Tomioka-higashi, Kanazawa-ku, Yokohama, 236-0051, Japan.
⁸ Department of Respiratory Medicine, Kobe City Medical Center West Hospital, 2-4 Ichiban-cho, Nagata-ku, Kobe, 653-0013, Japan.
⁹ Nippon Medical School, Graduate School of Pulmonary Medicine, 1-1-5 Sendagi, Bunkyo-ku, Tokyo, 113-8603, Japan.
¹⁰ Clinical Research Center, National Hospital Organization Kinki-Chuo Chest Medical Center, 1180 Nagasone-cho, Kita-ku, Sakai, 591-8555, Japan.
¹¹ Department of Respiratory Medicine, Hiroshima Prefectural Hospital, 1-5-54 Ujina-kanda, Minami-ku, Hiroshima, 734-8530, Japan.
¹² Department of Respiratory Medicine, National Hospital Organization Ibarakihigashi National Hospital, 825 Terunuma, Tokai-mura, Naka-gun, 319-1113, Ibaraki, Japan.
¹³ Division of Pulmonary Medicine, Department of Medicine, Tokai University School of Medicine, 143 Shimokasuya, Isehara, 259-1193, Japan.
¹⁴ Division of Medical Oncology and Respiratory Medicine, Department of Internal Medicine, Shimane University, 89-1 Enya-cho, Izumo, 693-8501, Japan.
¹⁵ Department of Respiratory Medicine, National Hospital Organization, Tenryu Hospital, 4201-2 Oro, Hamana-ku, Hamamatsu, 434-8511, Japan.
¹⁶ Department of Respiratory Center, Toranomon Hospital, 2-2-2 Toranomon, Minato-ku, Tokyo, 105-8470, Japan.
¹⁷ Department of Respiratory Medicine, Kobe City Medical Center General Hospital, Minatojima-Minamimachi, Chyuo-ku, Kobe, 650-0047, Japan.
¹⁸ Department of Respiratory Medicine, Institute of Science Tokyo Hospital, 1-5-45 Yushima, Bunkyo-ku, Tokyo, 113-8519, Japan.
¹⁹ Department of Respiratory Medicine and Allergology, Sapporo Medical University School of Medicine, 16-291 Minami1jyo-nishi, Tyuo-ku, Sapporo, 060-8543, Japan.
²⁰ Department of Respiratory Medicine, Kyorin University School of Medicine, 6-20-2 Shinkawa, Mitaka, Tokyo, 181-8611, Japan.
²¹ Research Institute for Diseases of the Chest, Graduate School of Medical Sciences, Fukuoka University Hospital, 7-45-1 Nanakuma, Jyonan-ku, Fukuoka, 814-0180, Japan.
²² Department of Analytic Human Pathology, Nippon Medical School, 1-1-5 Sendagi, Bunkyo-ku, Tokyo, 113-8603, Japan.
²³ Department of Pathology Informatics, Nagasaki University Graduate School of Biomedical Sciences, 1-12-4 Sakamoto, Nagasaki, 852-8523, Japan.
²⁴ Department of Radiology, Faculty of Medicine, Saga University, 5-1-1 Nabeshima, Saga, 849-8501, Japan.
²⁵ Department of Radiology, Kurume University School of Medicine, 67 Asahimachi, Kurume, 830-0011, Japan.
²⁶ Department of Radiology, National Hospital Organization Kinki-Chuo Chest Medical Center, 1180 Nagasone-cho, Kita-ku, Sakai, 591-8555, Japan.
²⁷ Department of Respirology, Graduate School of Medicine, Chiba University, 1-8-1 Inohana, Chyuo-ku, Chiba, 260-8677, Japan.
²⁸ Department of Respiratory Medicine, Toho University Omori Medical Center, 6-11-1 Omori-nishi, Ota-ku, Tokyo, 143-8541, Japan.
²⁹ Departments of Respiratory Medicine and Rheumatology, Graduate School of Biomedical Sciences, Tokushima University, 3-18-15 Kuramoto-cho, Tokushima, 770-8503, Japan.
³⁰ Departments of Molecular and Internal Medicine, Graduate School of Biomedical & Health Sciences, Hiroshima University, 1-2-3 Kasumi, Minami-ku, Hiroshima, 734-8551, Japan.
³¹ Department of Respiratory Medicine, Fujita Health University, 1-98 Dengakugakubo, Kutsukake-cho, Toyoake, 470-1192, Japan.
³² Department of Biomedical Statistics and Bioinformatics, Kyoto University, Yoshidakonoe-cho, Sakyo-ku, Kyoto, 606-8501, Japan.
³³ Department of Healthcare Quality Assessment, The University of Tokyo, 4-6-1 Shirokanedai, Minato-ku, Tokyo, 108-8639, Japan.
³⁴ Departments of Health Policy and Management, School of Medicine, Keio University, 35 Shinanomachi, Shinjyuku-ku, Tokyo, 160-8582, Japan.
³⁵ Department of Respiratory Medicine, Saitama Medical University International Medical Center, 1397-1 Yamane, Hidaka, 350-1298, Japan.
³⁶ Tohoku University, 2-1 Seiryomachi, Aoba-ku, Sendai, 980-8575, Japan.

PMID: 40101437
DOI: 10.1016/j.resinv.2025.02.009

Free article

Observational Study

Prognostic prediction for newly diagnosed patients with idiopathic interstitial pneumonia: JIPS Registry (NEJ030)

Ryo Okuda et al. Respir Investig. 2025 May.

Free article

. 2025 May;63(3):365-372.

doi: 10.1016/j.resinv.2025.02.009. Epub 2025 Mar 17.

Authors

Collaborators

JIPS registry collaborators:
Machiko Arita, Masahito Ebina, Yasutsugu Fukushima, Yoshihiro Funaki, Yasuhiro Gon, Tomohiro Handa, Hiromichi Hara, Dai Hashimoto, Seishu Hashimoto, Kazuya Ichikado, Hidenori Ichiyasu, Kazuyoshi Imaizumi, Minoru Inomata, Hiroshi Ishimoto, Hiroyuki Ito, Katsuyoshi Iwabuchi, Michiko Kagajo, Motoyasu Kato, Tomoya Katsuta, Tetsuji Kawamura, Masahiro Kimura, Yuichiro Kimura, Tomoo Kishaba, Yasuhiko Koga, Yasuo Kohashi, Chiyoko Kono, Kei Morikawa, Hiroaki Nakagawa, Osamu Narumoto, Hirotsugu Ohkubo, Masaki Okamoto, Yoshitaka Oyamada, Keita Sakamoto, Tomoya Sasaki, Shintaro Sato, Yusuke Shikama, Yasuo Shimizu, Shinko Suzuki, Kageaki Taima, Kazufumi Takamatsu, Motoaki Tanigawa, Yoshinori Tanino, Osamu Nishiyama, Mikio Toyoshima, Kosuke Tsuruno, Akifumi Uchida, Hirokazu Urushiyama, Kazuhiro Usui, Satoshi Wasamoto, Yuko Waseda, Satoshi Watanabe, Hiroshi Yamamoto, Kazuhiro Yatera, Koshi Yokomura

Affiliations

¹ Department of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory Center, 6-16-1 Tomioka-higashi, Kanazawa-ku, Yokohama, 236-0051, Japan.
² Department of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory Center, 6-16-1 Tomioka-higashi, Kanazawa-ku, Yokohama, 236-0051, Japan. Electronic address: takaoguogu@gmail.com.
³ Division of Pulmonary Medicine, Department of Medicine, Jichi Medical University, 3311-1 Yakushiji, Shimotsuke, 329-0498, Japan.
⁴ Department of Respiratory Medicine and Allergy, Tosei General Hospital, 160 Nishioiwake-cho, Seto, 489-8642, Japan.
⁵ Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, 1-20-1 Handayama, Tyuo-ku, Hamamatsu, 431-3192, Japan.
⁶ Department of Radiology, Kansai Rosai Hospital, 3-1-69 Inabaso, Amagasaki, 660-8511, Japan.
⁷ Department of Radiology, Kanagawa Cardiovascular and Respiratory Center, 6-16-1 Tomioka-higashi, Kanazawa-ku, Yokohama, 236-0051, Japan.
⁸ Department of Respiratory Medicine, Kobe City Medical Center West Hospital, 2-4 Ichiban-cho, Nagata-ku, Kobe, 653-0013, Japan.
⁹ Nippon Medical School, Graduate School of Pulmonary Medicine, 1-1-5 Sendagi, Bunkyo-ku, Tokyo, 113-8603, Japan.
¹⁰ Clinical Research Center, National Hospital Organization Kinki-Chuo Chest Medical Center, 1180 Nagasone-cho, Kita-ku, Sakai, 591-8555, Japan.
¹¹ Department of Respiratory Medicine, Hiroshima Prefectural Hospital, 1-5-54 Ujina-kanda, Minami-ku, Hiroshima, 734-8530, Japan.
¹² Department of Respiratory Medicine, National Hospital Organization Ibarakihigashi National Hospital, 825 Terunuma, Tokai-mura, Naka-gun, 319-1113, Ibaraki, Japan.
¹³ Division of Pulmonary Medicine, Department of Medicine, Tokai University School of Medicine, 143 Shimokasuya, Isehara, 259-1193, Japan.
¹⁴ Division of Medical Oncology and Respiratory Medicine, Department of Internal Medicine, Shimane University, 89-1 Enya-cho, Izumo, 693-8501, Japan.
¹⁵ Department of Respiratory Medicine, National Hospital Organization, Tenryu Hospital, 4201-2 Oro, Hamana-ku, Hamamatsu, 434-8511, Japan.
¹⁶ Department of Respiratory Center, Toranomon Hospital, 2-2-2 Toranomon, Minato-ku, Tokyo, 105-8470, Japan.
¹⁷ Department of Respiratory Medicine, Kobe City Medical Center General Hospital, Minatojima-Minamimachi, Chyuo-ku, Kobe, 650-0047, Japan.
¹⁸ Department of Respiratory Medicine, Institute of Science Tokyo Hospital, 1-5-45 Yushima, Bunkyo-ku, Tokyo, 113-8519, Japan.
¹⁹ Department of Respiratory Medicine and Allergology, Sapporo Medical University School of Medicine, 16-291 Minami1jyo-nishi, Tyuo-ku, Sapporo, 060-8543, Japan.
²⁰ Department of Respiratory Medicine, Kyorin University School of Medicine, 6-20-2 Shinkawa, Mitaka, Tokyo, 181-8611, Japan.
²¹ Research Institute for Diseases of the Chest, Graduate School of Medical Sciences, Fukuoka University Hospital, 7-45-1 Nanakuma, Jyonan-ku, Fukuoka, 814-0180, Japan.
²² Department of Analytic Human Pathology, Nippon Medical School, 1-1-5 Sendagi, Bunkyo-ku, Tokyo, 113-8603, Japan.
²³ Department of Pathology Informatics, Nagasaki University Graduate School of Biomedical Sciences, 1-12-4 Sakamoto, Nagasaki, 852-8523, Japan.
²⁴ Department of Radiology, Faculty of Medicine, Saga University, 5-1-1 Nabeshima, Saga, 849-8501, Japan.
²⁵ Department of Radiology, Kurume University School of Medicine, 67 Asahimachi, Kurume, 830-0011, Japan.
²⁶ Department of Radiology, National Hospital Organization Kinki-Chuo Chest Medical Center, 1180 Nagasone-cho, Kita-ku, Sakai, 591-8555, Japan.
²⁷ Department of Respirology, Graduate School of Medicine, Chiba University, 1-8-1 Inohana, Chyuo-ku, Chiba, 260-8677, Japan.
²⁸ Department of Respiratory Medicine, Toho University Omori Medical Center, 6-11-1 Omori-nishi, Ota-ku, Tokyo, 143-8541, Japan.
²⁹ Departments of Respiratory Medicine and Rheumatology, Graduate School of Biomedical Sciences, Tokushima University, 3-18-15 Kuramoto-cho, Tokushima, 770-8503, Japan.
³⁰ Departments of Molecular and Internal Medicine, Graduate School of Biomedical & Health Sciences, Hiroshima University, 1-2-3 Kasumi, Minami-ku, Hiroshima, 734-8551, Japan.
³¹ Department of Respiratory Medicine, Fujita Health University, 1-98 Dengakugakubo, Kutsukake-cho, Toyoake, 470-1192, Japan.
³² Department of Biomedical Statistics and Bioinformatics, Kyoto University, Yoshidakonoe-cho, Sakyo-ku, Kyoto, 606-8501, Japan.
³³ Department of Healthcare Quality Assessment, The University of Tokyo, 4-6-1 Shirokanedai, Minato-ku, Tokyo, 108-8639, Japan.
³⁴ Departments of Health Policy and Management, School of Medicine, Keio University, 35 Shinanomachi, Shinjyuku-ku, Tokyo, 160-8582, Japan.
³⁵ Department of Respiratory Medicine, Saitama Medical University International Medical Center, 1397-1 Yamane, Hidaka, 350-1298, Japan.
³⁶ Tohoku University, 2-1 Seiryomachi, Aoba-ku, Sendai, 980-8575, Japan.

PMID: 40101437
DOI: 10.1016/j.resinv.2025.02.009

Abstract

Background: Prognostic factors in patients with newly diagnosed idiopathic interstitial pneumonia (IIP) have rarely been analyzed using prospective data. This study investigated prognostic factors in patients with IIP.

Methods: Central interstitial lung disease (ILD) experts established the diagnoses for fibrotic ILD. Prognostic factors using baseline data, including the pathological confidence level of usual interstitial pneumonia (UIP) assessed on a 0%-100% linear analog scale by high-resolution CT (HRCT), pulmonary function tests, and patient-reported outcomes were investigated.

Results: Overall, 866 eligible patients were registered. Patients with unclassifiable idiopathic interstitial pneumonia (n = 272) survived longer than those with idiopathic pulmonary fibrosis (IPF) (n = 469) (hazard ratio [HR] = 0.67; [95% confidence interval [CI]: 0.47-0.95]; P = 0.022); however, IPF as IIPs classification was not a significant prognostic factor at diagnosis (P = 0.577). UIP pattern on HRCT, age, body mass index, forced vital capacity, diffusing capacity of the lungs for carbon monoxide, and St. George's Respiratory Questionnaire were risk factors for survival (P < 0.05). Patients with proposed progressive pulmonary fibrosis (PPF) had poorer prognoses than those without proposed PPF (HR = 5.63; [95% CI: 3.17-10.00]; P < 0.001). Patients with progressive fibrosing ILD (PF-ILD) had poorer prognoses than those without PF-ILD (HR = 7.85; [95% CI: 3.38-18.3]; P < 0.001).

Conclusions: A prospective registry of patients with newly diagnosed IIP provided evidence that the UIP pattern on HRCT by analog scale was a prognostic predictor. Proposed PPF and PF-ILD were valuable for discriminating prognosis. (JIPS Registry, ClinTrials.gov, NCT03041623).

Keywords: Idiopathic pulmonary fibrosis; Interstitial lung disease; Progressive fibrosing interstitial lung disease; Progressive pulmonary fibrosis; Usual interstitial pneumonia.

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Conflict of interest statement

Declaration of competing interest T. Ogura has received honoraria from Nippon Boehringer Ingelheim Co., Ltd. S. Hisata has received honoraria from Nippon Boehringer Ingelheim Co., Ltd. T. Baba has received honoraria from Nippon Boehringer Ingelheim Co., Ltd. Y. Kondoh has received honoraria from Nippon Boehringer Ingelheim Co., Ltd. T. Suda has received honoraria and research funding from Nippon Boehringer Ingelheim Co., Ltd. and AstraZeneca K.K. T. Johkoh has received honoraria from Nippon Boehringer Ingelheim Co., Ltd., AstraZeneca K.K., and Kyorin Pharmaceutical Co., Ltd. T. Iwasawa has received research funding from Canon Medical systems Corp. H. Tomioka has received honoraria from Nippon Boehringer Ingelheim Co., Ltd. M. Bando has received honoraria from Nippon Boehringer Ingelheim Co., Ltd. A. Azuma has received honoraria, travel fees, gifts, and others from Nippon Boehringer Ingelheim Co., Ltd. Y. Inoue has received honoraria from Nippon Boehringer Ingelheim Co., Ltd. N. Ishikawa has received honoraria from Nippon Boehringer Ingelheim Co., Ltd. Y. Nakamura has received honoraria from Nippon Boehringer Ingelheim Co., Ltd. A. Miyamoto has received honoraria from Nippon Boehringer Ingelheim Co., Ltd. K. Tomii has received honoraria from Nippon Boehringer Ingelheim Co., Ltd., Teijin Pharma Ltd., and Teijin Healthcare. Y. Miyazaki has received honoraria, research funding, subsidies, and donations from Nippon Boehringer Ingelheim Co., Ltd. H. Chiba has received honoraria from Nippon Boehringer Ingelheim Co., Ltd. H. Ishii has received honoraria from Nippon Boehringer Ingelheim Co., Ltd., AstraZeneca K.K., KYORIN Pharmaceutical Co., Ltd., and GlaxoSmithKline K.K. N. Hamada has received research funding from Nippon Boehringer Ingelheim Co., Ltd. J. Fukuoka has stocks of Pathology Institute Corp., and N Lab. J. Fukuoka also has received manuscript fees from Ibex. K. Fujimoto has received research funding from Mikron K.K. T. Suzuki has received honoraria from Nippon Boehringer Ingelheim Co., Ltd., and AstraZeneca K.K. Y. Nishioka has received honoraria from Nippon Boehringer Ingelheim Co., Ltd. N. Hattori has received honoraria from Nippon Boehringer Ingelheim Co., Ltd., and Shionogi & Co., Ltd. H. Miyata has received research funding from National Clinical Database. K. Hagiwara has received advisory board fees, patent royalties, and licensing fees from Eiken Chemical Co., Ltd., and LSI Medience. K. Hagiwara also has received honoraria, research funding, subsidies, and donations from Eiken Chemical Co., Ltd., AstraZeneca K.K., Chugai pharmaceutical Co., Ltd., Taiho Pharmaceutical Co., Ltd., and Ono Pharmaceutical Co., Ltd. R. Okuda, N. Arai, T. Takihara, M. Hamaguchi, T. Arai, Y. Terasaki, F. Sakai, R. Egashira, H. Sumikawa, S. Sakamoto, N. Hashimoto, S. Morita, N. Ichihara, K. Kobayashi and T. Nukiwa have no competing interests.

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Prognostic prediction for newly diagnosed patients with idiopathic interstitial pneumonia: JIPS Registry (NEJ030)

Collaborators

Affiliations

Prognostic prediction for newly diagnosed patients with idiopathic interstitial pneumonia: JIPS Registry (NEJ030)

Authors

Collaborators

Affiliations

Abstract

Conflict of interest statement

Publication types

MeSH terms

Associated data

LinkOut - more resources

Full Text Sources

Medical