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

Authors :
Huapaya JA
Boulougoura A
Fried J
Mesdaghinia S
Culotta BJ
Carson S
Bergquist PJ
Krishnan P
Wang H
Reichner C
Steen V
Source :
Clinical and experimental rheumatology [Clin Exp Rheumatol] 2023 Jan; Vol. 41 (1), pp. 15-23. Date of Electronic Publication: 2022 Mar 30.
Publication Year :
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.<br />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.<br />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.<br />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.

Details

Language :
English
ISSN :
0392-856X
Volume :
41
Issue :
1
Database :
MEDLINE
Journal :
Clinical and experimental rheumatology
Publication Type :
Academic Journal
Accession number :
35383557
Full Text :
https://doi.org/10.55563/clinexprheumatol/tdueis