PMID- 38142756
OWN - NLM
STAT- MEDLINE
DCOM- 20240108
LR  - 20240116
IS  - 1532-3064 (Electronic)
IS  - 0954-6111 (Linking)
VI  - 221
DP  - 2024 Jan
TI  - Characteristics and risk factors of interstitial pneumonia with autoimmune 
      features.
PG  - 107500
LID - S0954-6111(23)00388-8 [pii]
LID - 10.1016/j.rmed.2023.107500 [doi]
AB  - BACKGROUND: Interstitial pneumonia with autoimmune features (IPAF) has features 
      of connective tissue disease-associated interstitial lung disease (CTD-ILD), but 
      without meeting criteria for a specific CTD. We compared baseline 
      characteristics, survival, and response to treatment of IPAF to both CTD-ILD and 
      unclassifiable ILD. METHODS: Measurements were extracted from a prospective 
      registry. Baseline features and survival were compared in IPAF against both 
      CTD-ILD and unclassifiable ILD. Linear trajectory of lung function decline 
      (%-predicted forced vital capacity [FVC%] and diffusion capacity of the lung for 
      carbon monoxide [DLCO%]) before and after initiation of mycophenolate or 
      azathioprine were compared in IPAF against both CTD-ILD and unclassifiable ILD 
      using linear mixed models. RESULTS: Compared to CTD-ILD (n = 1240), patients with 
      IPAF (n = 128) were older, more frequently male, and had greater smoking history. 
      Compared to unclassifiable ILD (n = 665), patients with IPAF were younger, more 
      frequently female, and had worse baseline lung function. IPAF had higher 
      mortality compared to CTD-ILD and similar risk of mortality compared to 
      unclassifiable ILD. Mycophenolate initiation was associated with stabilization of 
      FVC% and DLCO% in all ILD subtypes except for FVC% in patients with IPAF, and 
      azathioprine initiation with stabilization of FVC% and DLCO% in all ILD subtypes 
      except for FVC% decline in IPAF and DLCO% decline in CTD-ILD. CONCLUSION: 
      Patients with IPAF had worse survival compared to those with CTD-ILD and similar 
      mortality to unclassifiable ILD, with treatment being associated with 
      stabilization in lung function in all three ILDs. It is uncertain whether IPAF 
      should be considered a distinct ILD diagnostic subgroup.
CI  - Copyright (c) 2024 Elsevier Ltd. All rights reserved.
FAU - Vahidy, Sana
AU  - Vahidy S
AD  - Department of Medicine, University of British Columbia, Vancouver, BC, Canada; 
      Centre for Heart Lung Innovation, St. Paul's Hospital, Vancouver, BC, Canada.
FAU - Agyeman, Jonathan
AU  - Agyeman J
AD  - Department of Statistics, University of British Columbia, Vancouver, BC, Canada.
FAU - Zheng, Boyang
AU  - Zheng B
AD  - Department of Medicine, University of British Columbia, Vancouver, BC, Canada; 
      Division of Rheumatology, McGill University, Montreal, QC, Canada.
FAU - Donohoe, Kathryn
AU  - Donohoe K
AD  - Department of Medicine, University of British Columbia, Vancouver, BC, Canada.
FAU - Hambly, Nathan
AU  - Hambly N
AD  - Department of Medicine, McMaster University, Hamilton, ON, Canada.
FAU - Johannson, Kerri A
AU  - Johannson KA
AD  - Department of Medicine, University of Calgary, Calgary, AB, Canada.
FAU - Assayag, Deborah
AU  - Assayag D
AD  - Department of Medicine, McGill University, Montreal, QC, Canada.
FAU - Fisher, Jolene H
AU  - Fisher JH
AD  - Department of Medicine, University of Toronto, Toronto, ON, Canada.
FAU - Manganas, Helene
AU  - Manganas H
AD  - Department of Medicine, Universite de Montreal, Montreal, QC, Canada.
FAU - Marcoux, Veronica
AU  - Marcoux V
AD  - Department of Medicine, University of Saskatchewan, Saskatoon, SK, Canada.
FAU - Khalil, Nasreen
AU  - Khalil N
AD  - Department of Medicine, University of British Columbia, Vancouver, BC, Canada.
FAU - Kolb, Martin
AU  - Kolb M
AD  - Department of Medicine, McMaster University, Hamilton, ON, Canada.
FAU - Ryerson, Christopher J
AU  - Ryerson CJ
AD  - Department of Medicine, University of British Columbia, Vancouver, BC, Canada; 
      Centre for Heart Lung Innovation, St. Paul's Hospital, Vancouver, BC, Canada. 
      Electronic address: chris.ryerson@hli.ubc.ca.
CN  - CARE-PF Investigators
FAU - Wong, Alyson W
AU  - Wong AW
AD  - Department of Medicine, University of British Columbia, Vancouver, BC, Canada; 
      Centre for Heart Lung Innovation, St. Paul's Hospital, Vancouver, BC, Canada.
FAU - Lok, Stacey
AU  - Lok S
AD  - Department of Medicine, University of Saskatchewan, Saskatoon, SK, Canada.
FAU - Morisset, Julie
AU  - Morisset J
AD  - Department of Medicine, Universite de Montreal, Montreal, QC, Canada.
FAU - Fell, Charlene D
AU  - Fell CD
AD  - Department of Medicine, University of Calgary, Calgary, AB, Canada.
FAU - Shapera, Shane
AU  - Shapera S
AD  - Department of Medicine, University of Toronto, Toronto, ON, Canada.
FAU - Gershon, Andrea S
AU  - Gershon AS
AD  - Department of Medicine, University of Toronto, Toronto, ON, Canada.
FAU - Cox, Gerard
AU  - Cox G
AD  - Department of Medicine, McMaster University, Hamilton, ON, Canada.
FAU - Halayko, Andrew J
AU  - Halayko AJ
AD  - Department of Medicine, University of Manitoba, Winnipeg, MB, Canada.
FAU - Sadatsafavi, Mohsen
AU  - Sadatsafavi M
AD  - Phamaceutical Sciences, University of British Columbia, Vancouver, BC, Canada.
FAU - Wilcox, Pearce G
AU  - Wilcox PG
AD  - Department of Medicine, University of British Columbia, Vancouver, BC, Canada.
FAU - To, Teresa
AU  - To T
AD  - Department of Medicine, University of Toronto, Toronto, ON, Canada.
LA  - eng
PT  - Journal Article
DEP - 20231222
PL  - England
TA  - Respir Med
JT  - Respiratory medicine
JID - 8908438
RN  - MRK240IY2L (Azathioprine)
RN  - 0 (Immunosuppressive Agents)
SB  - IM
MH  - Humans
MH  - Male
MH  - Female
MH  - Azathioprine/therapeutic use
MH  - *Lung Diseases, Interstitial/complications/drug therapy
MH  - Lung
MH  - *Connective Tissue Diseases/diagnosis
MH  - Immunosuppressive Agents/therapeutic use
MH  - Risk Factors
OTO - NOTNLM
OT  - Connective tissue disease
OT  - Connective tissue disease interstitial lung disease
OT  - Interstitial lung disease
OT  - Interstitial pneumonia with autoimmune features
OT  - Unclassifiable interstitial lung disease
COIS- Declaration of competing interest CJR, NK, KAJ, DA, HM, JF, MK, NH, SV report 
      personal fees from Boehringer Ingelheim. Conflict of interest: SV reports no 
      conflicts of interest relevant to this manuscript. Conflict of interest: JA 
      reports no conflicts of interest relevant to this manuscript. Conflict of 
      interest: BZ reports no conflicts of interest relevant to this manuscript. 
      Conflict of interest: K. Donohoe reports no conflicts of interest relevant to 
      this manuscript. Conflict of interest: N. Hambly reports grants from Roche and 
      Janssen; personal fees from Roche, Boehringer Ingelheim, GSK and Janssen. 
      Conflict of interest: K.A. Johannson reports grants from Three Lakes and 
      University Hospital Foundation. K.J. reports consulting fees from 
      Boehringer-Ingelheim, Hoffman-La Roche Ltd, Pliant Therapeutics, Three Lakes, 
      Thyron and Brainomix. Conflict of interest: D. Assayag reports no conflicts of 
      interest relevant to this manuscript. Conflict of interest: J. Fisher reports no 
      conflicts of interest relevant to this manuscript. Conflict of interest: V. 
      Marcoux reports personal fees from Boehringer Ingelheim Canada and Hoffman-La 
      Roche Ltd and Astra Zeneca, grants from the University of Saskatchewan, 
      Boehringer Ingelheim, Astra Zeneca and Hoffman-La Roche. VM reports consulting 
      fees from Boehringer Ingelheim Canada and Hoffman-La Roche Ltd. Conflict of 
      interest: H. Manganas reports grants from Boehringer Ingelheim Canada, Hoffmann 
      La Roche, Galapagos, BMS. Conflict of interest: N. Khalil reports no conflicts of 
      interest relevant to this manuscript. Conflict of interest: M. Kolb reports 
      consulting fees from Boehringer Ingelheim, Roche, Horizon, Cipla Abbvie, 
      Bellerophon, Algernon, CSL Behring, United Therapeutics, LabCorp, Structure 
      Therapeutics and Astra Zeneca. M. Kolb reports grants from Boehringer Ingelheim, 
      United Therapeutics and Structure Therapeutics. M. Kolb reports payment for 
      expert testimony from Roche. Conflict of interest: C.J. Ryerson reports grants 
      from Boehringer Ingelheim; consulting fees from Boehringer Ingelheim, Trevi 
      Therapeutics, Pliant Therapeutics, Astrazeneca and Veracyte.
EDAT- 2023/12/25 00:42
MHDA- 2024/01/08 06:43
CRDT- 2023/12/24 19:25
PHST- 2023/08/25 00:00 [received]
PHST- 2023/11/13 00:00 [revised]
PHST- 2023/12/11 00:00 [accepted]
PHST- 2024/01/08 06:43 [medline]
PHST- 2023/12/25 00:42 [pubmed]
PHST- 2023/12/24 19:25 [entrez]
AID - S0954-6111(23)00388-8 [pii]
AID - 10.1016/j.rmed.2023.107500 [doi]
PST - ppublish
SO  - Respir Med. 2024 Jan;221:107500. doi: 10.1016/j.rmed.2023.107500. Epub 2023 Dec 
      22.