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. 2024 Jan:221:107500.
doi: 10.1016/j.rmed.2023.107500. Epub 2023 Dec 22.

Characteristics and risk factors of interstitial pneumonia with autoimmune features

Sana Vahidy  1 , Jonathan Agyeman  2 , Boyang Zheng  3 , Kathryn Donohoe  4 , Nathan Hambly  5 , Kerri A Johannson  6 , Deborah Assayag  7 , Jolene H Fisher  8 , Helene Manganas  9 , Veronica Marcoux  10 , Nasreen Khalil  4 , Martin Kolb  5 , Christopher J Ryerson  11 ; CARE-PF Investigators; Alyson W Wong  1 , Stacey Lok  10 , Julie Morisset  9 , Charlene D Fell  6 , Shane Shapera  8 , Andrea S Gershon  8 , Gerard Cox  5 , Andrew J Halayko  12 , Mohsen Sadatsafavi  13 , Pearce G Wilcox  4 , Teresa To  8
Affiliations

Characteristics and risk factors of interstitial pneumonia with autoimmune features

Sana Vahidy et al. Respir Med. 2024 Jan.

Abstract

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.

Keywords: Connective tissue disease; Connective tissue disease interstitial lung disease; Interstitial lung disease; Interstitial pneumonia with autoimmune features; Unclassifiable interstitial lung disease.

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Conflict of interest statement

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.

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