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Characterisation of patients with interstitial pneumonia with autoimmune features

Justin M. Oldham, Ayodeji Adegunsoye, Eleanor Valenzi, Cathryn Lee, Leah Witt, Lena Chen, Aliya N. Husain, Steven Montner, Jonathan H. Chung, Vincent Cottin, Aryeh Fischer, Imre Noth, Rekha Vij, Mary E. Strek
European Respiratory Journal 2016 47: 1767-1775; DOI: 10.1183/13993003.01565-2015
Justin M. Oldham
1Dept of Medicine, Division of Pulmonary, Critical Care and Sleep Medicine, The University of California at Davis
8These authors contributed equally
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  • For correspondence: joldham@ucdavis.edu
Ayodeji Adegunsoye
2Dept of Medicine, Section of Pulmonary and Critical Care Medicine, The University of Chicago, Chicago, IL, USA
8These authors contributed equally
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Eleanor Valenzi
3Dept of Medicine, The University of Chicago, Chicago, IL, USA
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Cathryn Lee
3Dept of Medicine, The University of Chicago, Chicago, IL, USA
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Leah Witt
2Dept of Medicine, Section of Pulmonary and Critical Care Medicine, The University of Chicago, Chicago, IL, USA
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Lena Chen
2Dept of Medicine, Section of Pulmonary and Critical Care Medicine, The University of Chicago, Chicago, IL, USA
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Aliya N. Husain
4Dept of Pathology, The University of Chicago, Chicago, IL, USA
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Steven Montner
5Dept of Radiology, The University of Chicago, Chicago, IL, USA
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Jonathan H. Chung
5Dept of Radiology, The University of Chicago, Chicago, IL, USA
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Vincent Cottin
6Dept of Respiratory Medicine, Louis Pradel Hospital, Hospices Civils de Lyon, University Claude Bernard Lyon, Lyon, France
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Aryeh Fischer
7Dept of Medicine, Division of Rheumatology, University of Colorado, Denver, CO, USA
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Imre Noth
2Dept of Medicine, Section of Pulmonary and Critical Care Medicine, The University of Chicago, Chicago, IL, USA
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Rekha Vij
2Dept of Medicine, Section of Pulmonary and Critical Care Medicine, The University of Chicago, Chicago, IL, USA
9These authors contributed equally
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Mary E. Strek
2Dept of Medicine, Section of Pulmonary and Critical Care Medicine, The University of Chicago, Chicago, IL, USA
9These authors contributed equally
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This article has a correction. Please see:

  • “Characterisation of patients with interstitial pneumonia with autoimmune features.” Justin M. Oldham, Ayodeji Adegunsoye, Eleanor Valenzi, Cathryn Lee, Leah Witt, Lena Chen, Aliya N. Husain, Steven Montner, Jonathan H. Chung, Vincent Cottin, Aryeh Fischer, Imre Noth, Rekha Vij and Mary E. Strek. Eur Respir J 2016; 47: 1767–1775. - May 01, 2017

Abstract

Patients with interstitial lung disease (ILD) may have features of connective tissue disease (CTD), but lack findings diagnostic of a specific CTD. A recent European Respiratory Society/American Thoracic Society research statement proposed criteria for patients with interstitial pneumonia with autoimmune features (IPAF).

We applied IPAF criteria to patients with idiopathic interstitial pneumonia and undifferentiated CTD-ILD (UCTD). We then characterised the clinical, serological and morphological features of the IPAF cohort, compared outcomes to other ILD cohorts and validated individual IPAF domains using survival as an endpoint.

Of 422 patients, 144 met IPAF criteria. Mean age was 63.2 years with a slight female predominance. IPAF cohort survival was marginally better than patients with idiopathic pulmonary fibrosis, but worse than CTD-ILD. A non-usual interstitial pneumonia pattern was associated with improved survival, as was presence of the clinical domain. A modified IPAF cohort of those meeting the clinical domain and a radiographic or histological feature within the morphological domain displayed survival similar to those with CTD-ILD.

IPAF is common among patients with idiopathic interstitial pneumonia and UCTD. Specific IPAF features can identify subgroups with differential survival. Further research is needed to replicate these findings and determine whether patients meeting IPAF criteria benefit from immunosuppressive therapy.

Abstract

IPAF is common among patients with IIP and has distinct subgroups that demonstrate differential survival http://ow.ly/Z0ShD

Footnotes

  • Editorial comment in Eur Respir J 2016; 47: 1622–1624.

  • Support statement: This study was funded by the US Department of Health and Human Services National Institutes of Health, grant number: T32 HL007605. Funding information for this article has been deposited with FundRef.

  • Conflict of interests: Disclosures can be found alongside the online version of this article at erj.ersjournals.com

  • Received September 20, 2015.
  • Accepted February 22, 2016.
  • Copyright ©ERS 2016
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Characterisation of patients with interstitial pneumonia with autoimmune features
Justin M. Oldham, Ayodeji Adegunsoye, Eleanor Valenzi, Cathryn Lee, Leah Witt, Lena Chen, Aliya N. Husain, Steven Montner, Jonathan H. Chung, Vincent Cottin, Aryeh Fischer, Imre Noth, Rekha Vij, Mary E. Strek
European Respiratory Journal Jun 2016, 47 (6) 1767-1775; DOI: 10.1183/13993003.01565-2015

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Characterisation of patients with interstitial pneumonia with autoimmune features
Justin M. Oldham, Ayodeji Adegunsoye, Eleanor Valenzi, Cathryn Lee, Leah Witt, Lena Chen, Aliya N. Husain, Steven Montner, Jonathan H. Chung, Vincent Cottin, Aryeh Fischer, Imre Noth, Rekha Vij, Mary E. Strek
European Respiratory Journal Jun 2016, 47 (6) 1767-1775; DOI: 10.1183/13993003.01565-2015
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