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Long-term evaluation of pulmonary function and survival of patients with interstitial pneumonia with autoimmune features


1, 2, 3, 4, 5, 6, 7, 8, 9, 10, 11

 

  1. Department of Critical Care Medicine, National Institutes of Health, Bethesda, MD, USA.
  2. Division of Rheumatology and Clinical Immunology, Beth Israel Deaconess Medical Center, Boston, MA, USA.
  3. Division of Pulmonary, Critical Care and Sleep Medicine, MedStar Georgetown University Hospital, Washington, DC, USA.
  4. Division of Rheumatology, MedStar Georgetown University Hospital, Washington, DC, USA.
  5. Division of Pulmonary Diseases, Critical Care and Environmental Medicine, Tulane University School of Medicine, New Orleans, LA, USA.
  6. Division of Pulmonary and Critical Care Medicine, Johns Hopkins University School of Medicine, Baltimore, MD, USA.
  7. Department of Radiology, MedStar Georgetown University Hospital, Washington, DC, USA.
  8. Department of Radiology, MedStar Georgetown University Hospital, Washington, DC, USA.
  9. Department of Biostatistics and Biomedical Informatics. Medstar Health Research Institute, Hyattsville, MD, USA.
  10. Division of Pulmonary, Critical Care and Sleep Medicine, MedStar Georgetown University Hospital, Washington, DC, USA.
  11. Division of Rheumatology, MedStar Georgetown University Hospital, Washington, DC, USA. steenv@georgetown.edu

CER15376
2023 Vol.41, N°1
PI 0015, PF 0023
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PMID: 35383557 [PubMed]

Received: 28/11/2021
Accepted : 07/02/2022
In Press: 30/03/2022
Published: 23/01/2023

Abstract

OBJECTIVES:
Interstitial pneumonia with autoimmune features (IPAF) includes patients with interstitial lung disease with autoimmune features who do not meet criteria for a connective tissue disease (CTD). Previous studies showed a wide variation in the radiologic pattern, pulmonary function and prognosis but there is still limited data on longitudinal outcomes. We aim to describe the long-term pulmonary function, radiological patterns, and survival of IPAF patients and explore a classification based on CTD-like subgroups by using clinical/serologic data.
METHODS:
Retrospective analysis of IPAF patients who were sub-classified into six CTD-(like) subgroups: systemic lupus erythematosus-like, rheumatoid arthritis-like, Sjögren’s syndrome-like, scleroderma, myositis-like, and unclassifiable. Linear mixed-effect models were used to compare the change in percent-predicted forced vital capacity (FVC%), percent-predicted diffusion capacity (DLCO%), and six-minute walk distance (SMWD) over time; and survival in the entire cohort and according to CTD-like subgroups and radiological patterns.
RESULTS:
Fifty-nine patients fulfilled IPAF criteria. FVC%, DLCO%, and SMWD remained stable over time. There was no difference between usual interstitial pneumonia (UIP) versus non-UIP radiologic patterns. Thirty-five patients were sub-classified into CTD-like subgroups. Survival decreased from 79% at 60 months to 53% at 120 months in the entire cohort but was similar among CTD-like subgroups and radiological patterns.
CONCLUSIONS:
Long-term pulmonary function and six-minute walk test remained stable over 36 months in our IPAF cohort. Prognosis and pulmonary function in UIP had similar outcomes compared to non-UIP. Although 40% of IPAF patients could not be sub-classified, our exploratory subclassification stratified 60% of patients into a CTD-like subgroup.

DOI: https://doi.org/10.55563/clinexprheumatol/tdueis

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