PMID- 31272428
OWN - NLM
STAT- MEDLINE
DCOM- 20200108
LR  - 20200225
IS  - 1471-2466 (Electronic)
IS  - 1471-2466 (Linking)
VI  - 19
IP  - 1
DP  - 2019 Jul 4
TI  - Interstitial pneumonia with autoimmune features show better survival and less 
      exacerbations compared to idiopathic pulmonary fibrosis.
PG  - 120
LID - 10.1186/s12890-019-0868-9 [doi]
LID - 120
AB  - BACKGROUND: Patients with interstitial lung disease (ILD) who show features 
      related to autoimmunity without meeting criteria for a defined connective tissue 
      disease are categorized as interstitial pneumonia with autoimmune features 
      (IPAF). The present study compared clinical characteristics and clinical outcomes 
      of patients with IPAF to patients with connective tissue disease 
      related-interstitial lung disease (CTD-ILD) and patients with idiopathic 
      pulmonary fibrosis (IPF). METHODS: ILD patients who were consecutively enrolled 
      in a single institution ILD cohort between 2008 and 2015 were evaluated for the 
      study. Clinical data had been prospectively collected, while radiologic imaging 
      and pathologic findings were re-reviewed for the present study. RESULTS: Out of 
      305 patients with ILD, 54 (17.7%) patients met the classification of IPAF, 175 
      (57.4%) patients had IPF, and 76 (24.9%) patients were diagnosed with CTD-ILD. 
      Compared to IPF, incidences of acute exacerbations in 1,3 and 5 years were 
      significantly less in the IPAF group (p = 0.022, p = 0.026 and p = 0.007, 
      respectively). From multivariate analysis for mortality, age (p = 0.034, HR 
      1.022, 95% CI: 1.002-1.044), FVC (p < 0.001, HR 0.970, 95% CI: 0.955-0.984), ILD 
      exacerbation (p = 0.001, HR 2.074, 95% CI: 1.366-3.148), and ILD type (p = 0.047, 
      HR 0.436, 95% CI: 0.192-0.984 (IPAF vs IPF), respectively) showed significant 
      association. CONCLUSIONS: Compared to the other ILD groups, IPAF showed distinct 
      clinical characteristics. The IPAF group showed better survival and less episodes 
      of exacerbation when compared to the IPF group.
FAU - Lim, Jeong Uk
AU  - Lim JU
AD  - Division of Pulmonary and Critical Care Medicine, Department of Medicine, The 
      Armed Forces Capital Hospital, Seongnam, South Korea.
AD  - Division of Pulmonary, Allergy and Critical Care Medicine, Department of Internal 
      Medicine, Bucheon St. Mary's Hospital, College of Medicine, The Catholic 
      University of Korea, 327, Sosa-ro, Bucheon-si, Gyeonggi-do, 14647, Republic of 
      Korea.
FAU - Gil, Bo Mi
AU  - Gil BM
AD  - Department of Radiology, Bucheon St Mary's Hospital, College of Medicine, The 
      Catholic University of Korea, Bucheon, South Korea.
FAU - Kang, Hye Seon
AU  - Kang HS
AD  - Division of Pulmonary, Allergy and Critical Care Medicine, Department of Internal 
      Medicine, Bucheon St. Mary's Hospital, College of Medicine, The Catholic 
      University of Korea, 327, Sosa-ro, Bucheon-si, Gyeonggi-do, 14647, Republic of 
      Korea.
FAU - Oh, Jongyeol
AU  - Oh J
AD  - Division of Pulmonary, Allergy and Critical Care Medicine, Department of Internal 
      Medicine, Bucheon St. Mary's Hospital, College of Medicine, The Catholic 
      University of Korea, 327, Sosa-ro, Bucheon-si, Gyeonggi-do, 14647, Republic of 
      Korea.
FAU - Kim, Yong Hyun
AU  - Kim YH
AD  - Division of Pulmonary, Allergy and Critical Care Medicine, Department of Internal 
      Medicine, Bucheon St. Mary's Hospital, College of Medicine, The Catholic 
      University of Korea, 327, Sosa-ro, Bucheon-si, Gyeonggi-do, 14647, Republic of 
      Korea. kyh30med@catholic.ac.kr.
FAU - Kwon, Soon Seog
AU  - Kwon SS
AD  - Division of Pulmonary, Allergy and Critical Care Medicine, Department of Internal 
      Medicine, Bucheon St. Mary's Hospital, College of Medicine, The Catholic 
      University of Korea, 327, Sosa-ro, Bucheon-si, Gyeonggi-do, 14647, Republic of 
      Korea.
LA  - eng
PT  - Comparative Study
PT  - Journal Article
DEP - 20190704
PL  - England
TA  - BMC Pulm Med
JT  - BMC pulmonary medicine
JID - 100968563
SB  - IM
MH  - Aged
MH  - Aged, 80 and over
MH  - *Autoimmunity
MH  - Connective Tissue Diseases/*complications/immunology
MH  - Databases, Factual
MH  - Female
MH  - Humans
MH  - Idiopathic Pulmonary Fibrosis/*mortality
MH  - Incidence
MH  - Lung Diseases, Interstitial/etiology/*mortality
MH  - Male
MH  - Middle Aged
MH  - Multivariate Analysis
MH  - Prospective Studies
MH  - Republic of Korea/epidemiology
MH  - Survival Analysis
MH  - Tomography, X-Ray Computed
PMC - PMC6610995
OTO - NOTNLM
OT  - Connective tissue disease
OT  - Idiopathic pulmonary fibrosis
OT  - Interstitial lung disease
OT  - Interstitial pneumonia with autoimmune features
COIS- The authors declare that they have no competing interests.
EDAT- 2019/07/06 06:00
MHDA- 2020/01/09 06:00
PMCR- 2019/07/04
CRDT- 2019/07/06 06:00
PHST- 2019/02/15 00:00 [received]
PHST- 2019/05/30 00:00 [accepted]
PHST- 2019/07/06 06:00 [entrez]
PHST- 2019/07/06 06:00 [pubmed]
PHST- 2020/01/09 06:00 [medline]
PHST- 2019/07/04 00:00 [pmc-release]
AID - 10.1186/s12890-019-0868-9 [pii]
AID - 868 [pii]
AID - 10.1186/s12890-019-0868-9 [doi]
PST - epublish
SO  - BMC Pulm Med. 2019 Jul 4;19(1):120. doi: 10.1186/s12890-019-0868-9.