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Respiratory investigation
Mar 17, 2025;63 (3): 365-372.

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

Ryo Okuda, Takashi Ogura, Shu Hisata, Tomohisa Baba, Yasuhiro Kondoh, Takafumi Suda, Takeshi Johkoh, Tae Iwasawa, Hiromi Tomioka, Masashi Bando, Arata Azuma, Yoshikazu Inoue, Nobuhisa Ishikawa, Naoki Arai, Takahisa Takihara, Megumi Hamaguchi, Toru Arai, Yutaro Nakamura, Atsushi Miyamoto, Keisuke Tomii, Yasunari Miyazaki, Hirofumi Chiba, Haruyuki Ishii, Naoki Hamada, Yasuhiro Terasaki, Junya Fukuoka, Fumikazu Sakai, Ryoko Egashira, Kiminori Fujimoto, Hiromitsu Sumikawa, Takuji Suzuki, Susumu Sakamoto, Yasuhiko Nishioka, Noboru Hattori, Naozumi Hashimoto, Satoshi Morita, Nao Ichihara, Hiroaki Miyata, Koichi Hagiwara, Kunihiko Kobayashi, Toshihiro Nukiwa, JIPS registry collaborators
Author Information
  1. Ryo Okuda: Department of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory Center, 6-16-1 Tomioka-higashi, Kanazawa-ku, Yokohama, 236-0051, Japan.
  2. Takashi Ogura: 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. Shu Hisata: Division of Pulmonary Medicine, Department of Medicine, Jichi Medical University, 3311-1 Yakushiji, Shimotsuke, 329-0498, Japan.
  4. Tomohisa Baba: Department of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory Center, 6-16-1 Tomioka-higashi, Kanazawa-ku, Yokohama, 236-0051, Japan.
  5. Yasuhiro Kondoh: Department of Respiratory Medicine and Allergy, Tosei General Hospital, 160 Nishioiwake-cho, Seto, 489-8642, Japan.
  6. Takafumi Suda: Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, 1-20-1 Handayama, Tyuo-ku, Hamamatsu, 431-3192, Japan.
  7. Takeshi Johkoh: Department of Radiology, Kansai Rosai Hospital, 3-1-69 Inabaso, Amagasaki, 660-8511, Japan.
  8. Tae Iwasawa: Department of Radiology, Kanagawa Cardiovascular and Respiratory Center, 6-16-1 Tomioka-higashi, Kanazawa-ku, Yokohama, 236-0051, Japan.
  9. Hiromi Tomioka: Department of Respiratory Medicine, Kobe City Medical Center West Hospital, 2-4 Ichiban-cho, Nagata-ku, Kobe, 653-0013, Japan.
  10. Masashi Bando: Division of Pulmonary Medicine, Department of Medicine, Jichi Medical University, 3311-1 Yakushiji, Shimotsuke, 329-0498, Japan.
  11. Arata Azuma: Nippon Medical School, Graduate School of Pulmonary Medicine, 1-1-5 Sendagi, Bunkyo-ku, Tokyo, 113-8603, Japan.
  12. Yoshikazu Inoue: Clinical Research Center, National Hospital Organization Kinki-Chuo Chest Medical Center, 1180 Nagasone-cho, Kita-ku, Sakai, 591-8555, Japan.
  13. Nobuhisa Ishikawa: Department of Respiratory Medicine, Hiroshima Prefectural Hospital, 1-5-54 Ujina-kanda, Minami-ku, Hiroshima, 734-8530, Japan.
  14. Naoki Arai: Department of Respiratory Medicine, National Hospital Organization Ibarakihigashi National Hospital, 825 Terunuma, Tokai-mura, Naka-gun, 319-1113, Ibaraki, Japan.
  15. Takahisa Takihara: Division of Pulmonary Medicine, Department of Medicine, Tokai University School of Medicine, 143 Shimokasuya, Isehara, 259-1193, Japan.
  16. Megumi Hamaguchi: Division of Medical Oncology and Respiratory Medicine, Department of Internal Medicine, Shimane University, 89-1 Enya-cho, Izumo, 693-8501, Japan.
  17. Toru Arai: Clinical Research Center, National Hospital Organization Kinki-Chuo Chest Medical Center, 1180 Nagasone-cho, Kita-ku, Sakai, 591-8555, Japan.
  18. Yutaro Nakamura: Department of Respiratory Medicine, National Hospital Organization, Tenryu Hospital, 4201-2 Oro, Hamana-ku, Hamamatsu, 434-8511, Japan.
  19. Atsushi Miyamoto: Department of Respiratory Center, Toranomon Hospital, 2-2-2 Toranomon, Minato-ku, Tokyo, 105-8470, Japan.
  20. Keisuke Tomii: Department of Respiratory Medicine, Kobe City Medical Center General Hospital, Minatojima-Minamimachi, Chyuo-ku, Kobe, 650-0047, Japan.
  21. Yasunari Miyazaki: Department of Respiratory Medicine, Institute of Science Tokyo Hospital, 1-5-45 Yushima, Bunkyo-ku, Tokyo, 113-8519, Japan.
  22. Hirofumi Chiba: Department of Respiratory Medicine and Allergology, Sapporo Medical University School of Medicine, 16-291 Minami1jyo-nishi, Tyuo-ku, Sapporo, 060-8543, Japan.
  23. Haruyuki Ishii: Department of Respiratory Medicine, Kyorin University School of Medicine, 6-20-2 Shinkawa, Mitaka, Tokyo, 181-8611, Japan.
  24. Naoki Hamada: 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.
  25. Yasuhiro Terasaki: Department of Analytic Human Pathology, Nippon Medical School, 1-1-5 Sendagi, Bunkyo-ku, Tokyo, 113-8603, Japan.
  26. Junya Fukuoka: Department of Pathology Informatics, Nagasaki University Graduate School of Biomedical Sciences, 1-12-4 Sakamoto, Nagasaki, 852-8523, Japan.
  27. Fumikazu Sakai: Department of Radiology, Kanagawa Cardiovascular and Respiratory Center, 6-16-1 Tomioka-higashi, Kanazawa-ku, Yokohama, 236-0051, Japan.
  28. Ryoko Egashira: Department of Radiology, Faculty of Medicine, Saga University, 5-1-1 Nabeshima, Saga, 849-8501, Japan.
  29. Kiminori Fujimoto: Department of Radiology, Kurume University School of Medicine, 67 Asahimachi, Kurume, 830-0011, Japan.
  30. Hiromitsu Sumikawa: Department of Radiology, National Hospital Organization Kinki-Chuo Chest Medical Center, 1180 Nagasone-cho, Kita-ku, Sakai, 591-8555, Japan.
  31. Takuji Suzuki: Department of Respirology, Graduate School of Medicine, Chiba University, 1-8-1 Inohana, Chyuo-ku, Chiba, 260-8677, Japan.
  32. Susumu Sakamoto: Department of Respiratory Medicine, Toho University Omori Medical Center, 6-11-1 Omori-nishi, Ota-ku, Tokyo, 143-8541, Japan.
  33. Yasuhiko Nishioka: Departments of Respiratory Medicine and Rheumatology, Graduate School of Biomedical Sciences, Tokushima University, 3-18-15 Kuramoto-cho, Tokushima, 770-8503, Japan.
  34. Noboru Hattori: Departments of Molecular and Internal Medicine, Graduate School of Biomedical & Health Sciences, Hiroshima University, 1-2-3 Kasumi, Minami-ku, Hiroshima, 734-8551, Japan.
  35. Naozumi Hashimoto: Department of Respiratory Medicine, Fujita Health University, 1-98 Dengakugakubo, Kutsukake-cho, Toyoake, 470-1192, Japan.
  36. Satoshi Morita: Department of Biomedical Statistics and Bioinformatics, Kyoto University, Yoshidakonoe-cho, Sakyo-ku, Kyoto, 606-8501, Japan.
  37. Nao Ichihara: Department of Healthcare Quality Assessment, The University of Tokyo, 4-6-1 Shirokanedai, Minato-ku, Tokyo, 108-8639, Japan.
  38. Hiroaki Miyata: Departments of Health Policy and Management, School of Medicine, Keio University, 35 Shinanomachi, Shinjyuku-ku, Tokyo, 160-8582, Japan.
  39. Koichi Hagiwara: Division of Pulmonary Medicine, Department of Medicine, Jichi Medical University, 3311-1 Yakushiji, Shimotsuke, 329-0498, Japan.
  40. Kunihiko Kobayashi: Department of Respiratory Medicine, Saitama Medical University International Medical Center, 1397-1 Yamane, Hidaka, 350-1298, Japan.
  41. Toshihiro Nukiwa: 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

Associated Data

ClinicalTrials.gov | NCT03041623
Journal Article
Article has an altmetric score of 1

Word Cloud

Created with Highcharts 10.0.0interstitialpatientspulmonaryfactorsidiopathicpneumoniafibrosisPrognosticnewlydiagnosedIIPprognosticlungdiseaseILDUIPHRCTPatients[95%P < 0PPFPF-ILDusingprospectivedatainvestigatedconfidenceanalogscaleIPFP = 0patterncapacityproposedprogressivepoorerprognoseswithoutCI:3001fibrosingJIPSRegistryProgressiveBACKGROUND:rarelyanalyzedstudyMETHODS:Centralexpertsestablisheddiagnosesfibroticbaselineincludingpathologicallevelusualassessed0%-100%linearhigh-resolutionCTfunctiontestspatient-reportedoutcomesRESULTS:Overall866eligibleregisteredunclassifiablen = 272survivedlongern = 469hazardratio[HR] = 067interval[CI]:047-095]022howeverIIPsclassificationsignificantfactordiagnosis577agebodymassindexforcedvitaldiffusinglungscarbonmonoxideStGeorge'sRespiratoryQuestionnairerisksurvival05HR = 56317-1000]HR = 78538-183]CONCLUSIONS:registryprovidedevidencepredictorProposedvaluablediscriminatingprognosisClinTrialsgovNCT03041623predictionpneumonia:NEJ030IdiopathicInterstitialUsual

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