PMID- 35383557
OWN - NLM
STAT- MEDLINE
DCOM- 20230125
LR  - 20231228
IS  - 0392-856X (Print)
IS  - 0392-856X (Linking)
VI  - 41
IP  - 1
DP  - 2023 Jan
TI  - Long-term evaluation of pulmonary function and survival of patients with 
      interstitial pneumonia with autoimmune features.
PG  - 15-23
LID - 10.55563/clinexprheumatol/tdueis [doi]
AB  - 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, Sjogren'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.
FAU - Huapaya, Julio A
AU  - Huapaya JA
AD  - Department of Critical Care Medicine, National Institutes of Health, Bethesda, 
      MD, USA.
FAU - Boulougoura, Afroditi
AU  - Boulougoura A
AD  - Division of Rheumatology and Clinical Immunology, Beth Israel Deaconess Medical 
      Center, Boston, MA, USA.
FAU - Fried, Julie
AU  - Fried J
AD  - Division of Pulmonary, Critical Care and Sleep Medicine, MedStar Georgetown 
      University Hospital, Washington, DC, USA.
FAU - Mesdaghinia, Sepehr
AU  - Mesdaghinia S
AD  - Division of Rheumatology, MedStar Georgetown University Hospital, Washington, DC, 
      USA.
FAU - Culotta, Brooks J
AU  - Culotta BJ
AD  - Division of Pulmonary Diseases, Critical Care and Environmental Medicine, Tulane 
      University School of Medicine, New Orleans, LA, USA.
FAU - Carson, Sara
AU  - Carson S
AD  - Division of Pulmonary and Critical Care Medicine, Johns Hopkins University School 
      of Medicine, Baltimore, MD, USA.
FAU - Bergquist, Peter J
AU  - Bergquist PJ
AD  - Department of Radiology, MedStar Georgetown University Hospital, Washington, DC, 
      USA.
FAU - Krishnan, Pranay
AU  - Krishnan P
AD  - Department of Radiology, MedStar Georgetown University Hospital, Washington, DC, 
      USA.
FAU - Wang, Haijun
AU  - Wang H
AD  - Department of Biostatistics and Biomedical Informatics. Medstar Health Research 
      Institute, Hyattsville, MD, USA.
FAU - Reichner, Cristina
AU  - Reichner C
AD  - Division of Pulmonary, Critical Care and Sleep Medicine, MedStar Georgetown 
      University Hospital, Washington, DC, USA.
FAU - Steen, Virginia
AU  - Steen V
AD  - Division of Rheumatology, MedStar Georgetown University Hospital, Washington, DC, 
      USA. steenv@georgetown.edu.
LA  - eng
PT  - Journal Article
DEP - 20220330
PL  - Italy
TA  - Clin Exp Rheumatol
JT  - Clinical and experimental rheumatology
JID - 8308521
SB  - IM
MH  - Humans
MH  - Retrospective Studies
MH  - *Lung Diseases, Interstitial
MH  - Lung
MH  - *Idiopathic Pulmonary Fibrosis
MH  - *Connective Tissue Diseases
MH  - *Autoimmune Diseases
EDAT- 2022/04/07 06:00
MHDA- 2023/01/26 06:00
CRDT- 2022/04/06 09:01
PHST- 2021/11/28 00:00 [received]
PHST- 2022/02/07 00:00 [accepted]
PHST- 2022/04/07 06:00 [pubmed]
PHST- 2023/01/26 06:00 [medline]
PHST- 2022/04/06 09:01 [entrez]
AID - 18161 [pii]
AID - 10.55563/clinexprheumatol/tdueis [doi]
PST - ppublish
SO  - Clin Exp Rheumatol. 2023 Jan;41(1):15-23. doi: 10.55563/clinexprheumatol/tdueis. 
      Epub 2022 Mar 30.