Characterisation of patients with interstitial pneumonia with autoimmune features.
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.
Dept of Medicine, Division of Pulmonary, Critical Care and Sleep Medicine, The University of California at Davis These authors contributed equally joldham@ucdavis.edu.
Justin M
JM
Oldham
Dept of Medicine, Section of Pulmonary and Critical Care Medicine, The University of Chicago, Chicago, IL, USA These authors contributed equally.
Ayodeji
A
Adegunsoye
Dept of Medicine, The University of Chicago, Chicago, IL, USA.
Eleanor
E
Valenzi
Dept of Medicine, The University of Chicago, Chicago, IL, USA.
Cathryn
C
Lee
Dept of Medicine, Section of Pulmonary and Critical Care Medicine, The University of Chicago, Chicago, IL, USA.
Leah
L
Witt
Dept of Medicine, Section of Pulmonary and Critical Care Medicine, The University of Chicago, Chicago, IL, USA.
Lena
L
Chen
Dept of Pathology, The University of Chicago, Chicago, IL, USA.
Aliya N
AN
Husain
Dept of Radiology, The University of Chicago, Chicago, IL, USA.
Steven
S
Montner
Dept of Radiology, The University of Chicago, Chicago, IL, USA.
Jonathan H
JH
Chung
Dept of Respiratory Medicine, Louis Pradel Hospital, Hospices Civils de Lyon, University Claude Bernard Lyon, Lyon, France.
Vincent
V
Cottin
Dept of Medicine, Division of Rheumatology, University of Colorado, Denver, CO, USA.
Aryeh
A
Fischer
Dept of Medicine, Section of Pulmonary and Critical Care Medicine, The University of Chicago, Chicago, IL, USA.
Imre
I
Noth
Dept of Medicine, Section of Pulmonary and Critical Care Medicine, The University of Chicago, Chicago, IL, USA These authors contributed equally.
Rekha
R
Vij
Dept of Medicine, Section of Pulmonary and Critical Care Medicine, The University of Chicago, Chicago, IL, USA These authors contributed equally.
Mary E
ME
Strek
D000368:Aged; D001327:Autoimmune Diseases / Q000175:diagnosis* / Q000276:immunology*; D001706:Biopsy; D015331:Cohort Studies; D003240:Connective Tissue Diseases / Q000175:diagnosis / Q000276:immunology; D005260:Female; D006801:Humans; D054988:Idiopathic Interstitial Pneumonias / Q000175:diagnosis* / Q000276:immunology*; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D053208:Kaplan-Meier Estimate; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D013997:Time Factors; D016896:Treatment Outcome
D007166:Immunosuppressive Agents