PMID- 34272335
OWN - NLM
STAT- MEDLINE
DCOM- 20220202
LR  - 20220202
IS  - 1468-3296 (Electronic)
IS  - 0040-6376 (Linking)
VI  - 77
IP  - 2
DP  - 2022 Feb
TI  - Prospective nationwide multicentre cohort study of the clinical significance of 
      autoimmune features in idiopathic interstitial pneumonias.
PG  - 143-153
LID - 10.1136/thoraxjnl-2020-216263 [doi]
AB  - BACKGROUND: Some patients with idiopathic interstitial pneumonia (IIP) show 
      autoimmune features. Interstitial pneumonia with autoimmune features (IPAF) was 
      recently proposed as a research concept in these patients. However, retrospective 
      studies reported conflicting results of its prognosis. Therefore, this study was 
      conducted to prospectively evaluate the clinical significance of autoimmune 
      features in patients with IIP. METHODS: This nationwide multicentre study 
      prospectively enrolled consecutive patients with IIP. At the diagnosis, we 
      systematically evaluated 63 features suggestive of connective tissue diseases 
      using a checklist including symptoms/signs and autoantibodies, which contained 
      most items of the IPAF criteria and followed up with the patients. Clinical 
      phenotypes were included in a cluster analysis. RESULTS: In 376 patients with IIP 
      enrolled, 70 patients (18.6%) met the IPAF criteria. The proportion of patients 
      with IPAF was significantly lower in idiopathic pulmonary fibrosis (IPF) than in 
      non-IPF (6.0% vs 24.3%, respectively). During a median observation period of 35 
      months, patients with IPAF more frequently developed systemic autoimmune diseases 
      and had less frequent acute exacerbation of IIPs than patients with non-IPAF. 
      IPAF diagnosis was significantly associated with better survival and was an 
      independent positive prognostic factor in total and patients with non-IPF. 
      Cluster analysis by similarity of clinical phenotypes identified a cluster in 
      which there was a higher number of women, and patients had more autoimmune 
      features and a better prognosis than other clusters. INTERPRETATION: These 
      observations suggest that some patients with IIP show autoimmune features with 
      distinct characteristics and favourable prognosis. However, we were not able to 
      determine the appropriate therapies for these patients.
CI  - (c) Author(s) (or their employer(s)) 2022. No commercial re-use. See rights and 
      permissions. Published by BMJ.
FAU - Enomoto, Noriyuki
AU  - Enomoto N
AD  - Second Division, Department of Internal Medicine, Hamamatsu University School of 
      Medicine, Hamamatsu, Japan norieno@hama-med.ac.jp.
FAU - Homma, Sakae
AU  - Homma S
AD  - Department of Advanced and Integrated Interstitial Lung Diseases Research, School 
      of Medicine, Toho University, Tokyo, Japan.
FAU - Inase, Naohiko
AU  - Inase N
AD  - Department of Respiratory Medicine, Tokyo Medical and Dental University, Tokyo, 
      Japan.
FAU - Kondoh, Yasuhiro
AU  - Kondoh Y
AD  - Department of Respiratory Medicine and Allergy, Tosei General Hospital, Aichi, 
      Japan.
FAU - Saraya, Takeshi
AU  - Saraya T
AD  - Department of Respiratory Medicine, Kyorin University School of Medicine, Tokyo, 
      Japan.
FAU - Takizawa, Hajime
AU  - Takizawa H
AD  - Department of Respiratory Medicine, Kyorin University School of Medicine, Tokyo, 
      Japan.
FAU - Inoue, Yoshikazu
AU  - Inoue Y
AD  - Clinical Research Center, National Hospital Organization Kinki-Chuo Chest Medical 
      Center, Osaka, Japan.
FAU - Ishii, Hiroshi
AU  - Ishii H
AD  - Department of Respiratory Medicine, Fukuoka University Hospital, Fukuoka, Japan.
FAU - Taguchi, Yoshio
AU  - Taguchi Y
AD  - Department of Respiratory Medicine, Tenri Hospital, Nara, Japan.
FAU - Izumi, Shinyu
AU  - Izumi S
AD  - Department of Respiratory Medicine, National Center for Global Health and 
      Medicine, Tokyo, Japan.
FAU - Yamano, Yasuhiko
AU  - Yamano Y
AD  - Department of Respiratory Medicine and Allergy, Tosei General Hospital, Aichi, 
      Japan.
FAU - Tanino, Yoshinori
AU  - Tanino Y
AD  - Department of Pulmonary Medicine, Fukushima Medical University, Fukushima, Japan.
FAU - Nishioka, Yasuhiko
AU  - Nishioka Y
AD  - Department of Respiratory Medicine and Rheumatology, Graduate School of 
      Biomedical Sciences, Tokushima University, Tokushima, Japan.
FAU - Toyoshima, Mikio
AU  - Toyoshima M
AD  - Department of Respiratory Medicine, Hamamatsu Rosai Hospital, Hamamatsu, Japan.
FAU - Yokomura, Koshi
AU  - Yokomura K
AD  - Department of Respiratory Medicine, Respiratory Disease Center, Seirei Mikatahara 
      General Hospital, Hamamatsu, Japan.
FAU - Imokawa, Shiro
AU  - Imokawa S
AD  - Department of Respiratory Medicine, Iwata City Hospital, Iwata, Japan.
FAU - Koshimizu, Naoki
AU  - Koshimizu N
AD  - Department of Respiratory Medicine, Fujieda Municipal General Hospital, Fujieda, 
      Japan.
FAU - Sano, Takehisa
AU  - Sano T
AD  - Department of Respiratory Medicine, Shizuoka City Shizuoka Hospital, Shizuoka, 
      Japan.
FAU - Akamatsu, Taisuke
AU  - Akamatsu T
AD  - Department of Respiratory Medicine, Shizuoka General Hospital, Shizuoka, Japan.
FAU - Mukae, Hiroshi
AU  - Mukae H
AD  - Department of Respiratory Medicine, Nagasaki University Graduate School of 
      Biomedical Sciences, Nagasaki, Japan.
FAU - Kato, Motoyasu
AU  - Kato M
AD  - Department of Respiratory Medicine, Juntendo University Graduate School of 
      Medicine, Tokyo, Japan.
FAU - Hamada, Naoki
AU  - Hamada N
AD  - Research Institute for Diseases of the Chest, Graduate School of Medical 
      Sciences, Kyushu University, Fukuoka, Japan.
FAU - Chiba, Hirofumi
AU  - Chiba H
AD  - Department of Respiratory Medicine and Allergology, Sapporo Medical University 
      School of Medicine, Sapporo, Japan.
FAU - Akagawa, Shinobu
AU  - Akagawa S
AD  - Center for Pulmonary Diseases, National Hospital Organization Tokyo National 
      Hospital, Tokyo, Japan.
FAU - Muro, Shigeo
AU  - Muro S
AUID- ORCID: 0000-0001-7452-9191
AD  - Department of Respiratory Medicine, Nara Medical University, Nara, Japan.
FAU - Uruga, Hironori
AU  - Uruga H
AD  - Department of Respiratory Medicine, Respiratory Center, Toranomon Hospital, 
      Tokyo, Japan.
FAU - Matsuda, Hiroyuki
AU  - Matsuda H
AD  - Department of Respiratory Medicine, Japanese Red Cross Shizuoka Hospital, 
      Shizuoka, Japan.
FAU - Kaida, Yusuke
AU  - Kaida Y
AD  - Department of Respiratory Medicine, Enshu Hospital, Hamamatsu, Japan.
FAU - Kanai, Miho
AU  - Kanai M
AD  - Respiratory and Allergy Medicine, National Hospital Organization Tenryu Hospital, 
      Hamamatsu, Japan.
FAU - Mori, Kazutaka
AU  - Mori K
AD  - Respiratory Medicine, Shizuoka City Shimizu Hospital, Shizuoka, Japan.
FAU - Masuda, Masafumi
AU  - Masuda M
AD  - Respiratory Medicine, Shizuoka City Shimizu Hospital, Shizuoka, Japan.
FAU - Hozumi, Hironao
AU  - Hozumi H
AUID- ORCID: 0000-0001-5439-1543
AD  - Second Division, Department of Internal Medicine, Hamamatsu University School of 
      Medicine, Hamamatsu, Japan.
FAU - Fujisawa, Tomoyuki
AU  - Fujisawa T
AD  - Second Division, Department of Internal Medicine, Hamamatsu University School of 
      Medicine, Hamamatsu, Japan.
FAU - Nakamura, Yutaro
AU  - Nakamura Y
AD  - Second Division, Department of Internal Medicine, Hamamatsu University School of 
      Medicine, Hamamatsu, Japan.
FAU - Ogawa, Noriyoshi
AU  - Ogawa N
AD  - Division of Immunology and Rheumatology, Department of Internal Medicine 3, 
      Hamamatsu University School of Medicine, Hamamatsu, Japan.
FAU - Suda, Takafumi
AU  - Suda T
AD  - Second Division, Department of Internal Medicine, Hamamatsu University School of 
      Medicine, Hamamatsu, Japan.
LA  - eng
SI  - UMIN-CTR/UMIN000010231
PT  - Journal Article
PT  - Multicenter Study
DEP - 20210716
PL  - England
TA  - Thorax
JT  - Thorax
JID - 0417353
SB  - IM
MH  - Female
MH  - Humans
MH  - *Idiopathic Interstitial Pneumonias
MH  - *Lung Diseases, Interstitial
MH  - Prospective Studies
MH  - Retrospective Studies
MH  - Tomography, X-Ray Computed
OTO - NOTNLM
OT  - connective tissue disease associated lung disease
OT  - idiopathic pulmonary fibrosis
OT  - interstitial fibrosis
COIS- Competing interests: YK has received personal fees from Asahi Kasei Pharma Corp., 
      from Boehringer Ingelheim Co., Ltd., from Janssen Pharmaceutical K.K., from Eisai 
      Inc., from Kyorin Pharmaceutical Co., Ltd., from Mitsubishi Tanabe Pharma, from 
      Novartis Pharma K.K., and from Shionogi & Co., outside the submitted work. YI has 
      received grants from Japanese ministry of Health, Labour, and Welfare, grants 
      from Japan Agency for Medical Research and Development, and lecture fee from 
      Boehringer Ingelheim and Shionogi outside the submitted work. YN has received 
      grants and personal fees from Nippon Boehringer Ingelheim Co., Ltd., grants and 
      personal fees from MSD K.K., grants and personal fees from Ono Pharmaceutical 
      Co., Ltd., grants and personal fees from Taiho Pharmaceutical Co., Ltd., grants 
      and personal fees from Chugai Pharmaceutical Co., Ltd., grants and personal fees 
      from Asahi Kaesi Pharma Corporation, grants and personal fees from Eli Lilly 
      Japan K.K., and grants from Bonac Corporation, outside the submitted work. HM has 
      received grants and personal fees from Daiichi Sankyo, grants and personal fees 
      from MSD, grants and personal fees from Sumitomo Dainippon Pharma, grants and 
      personal fees from Taisho Toyama Pharmaceutical, grants and personal fees from 
      Astellas Pharma, grants and personal fees from Boehringer Ingelheim, grants and 
      personal fees from Shionogi, grants from Taiho Pharmaceutical, grants from Ono 
      Pharmaceutical, grants from Takeda Pharmaceutical, grants from Eli Lilly Japan, 
      grants from Novartis Pharma, grants from Fujifilm Pharma, grants from Meiji Seika 
      Pharma, grants from Toyama Chemical, grants from Eisai, grants from Chugai 
      Pharmaceutical, personal fees from Pfizer, personal fees from Kyorin 
      Pharmaceutical, and personal fees from AstraZeneca, outside the submitted work. 
      TS has received lecture fees from Boehringer Ingelheim and Shionogi, outside the 
      submitted work.
EDAT- 2021/07/18 06:00
MHDA- 2022/02/03 06:00
CRDT- 2021/07/17 05:44
PHST- 2020/09/28 00:00 [received]
PHST- 2021/05/10 00:00 [accepted]
PHST- 2021/07/18 06:00 [pubmed]
PHST- 2022/02/03 06:00 [medline]
PHST- 2021/07/17 05:44 [entrez]
AID - thoraxjnl-2020-216263 [pii]
AID - 10.1136/thoraxjnl-2020-216263 [doi]
PST - ppublish
SO  - Thorax. 2022 Feb;77(2):143-153. doi: 10.1136/thoraxjnl-2020-216263. Epub 2021 Jul 
      16.