PMID- 35697042
OWN - NLM
STAT- MEDLINE
DCOM- 20220727
LR  - 20230927
IS  - 1536-7355 (Electronic)
IS  - 1076-1608 (Linking)
VI  - 28
IP  - 5
DP  - 2022 Aug 1
TI  - Identification and Prognosis of Patients With Interstitial Pneumonia With 
      Autoimmune Features.
PG  - 257-264
LID - 10.1097/RHU.0000000000001847 [doi]
AB  - BACKGROUND/OBJECTIVE: Patients classified as interstitial pneumonia with 
      autoimmune features (IPAF) have interstitial lung disease (ILD) and features of 
      autoimmunity but do not fulfill criteria for connective tissue diseases (CTDs). 
      Our goal was to identify patients classifiable as IPAF, CTD-ILD, and idiopathic 
      pulmonary fibrosis (IPF) from a preexisting pulmonary cohort and evaluate the 
      prognosis of patients with IPAF. METHODS: We reviewed the medical records of 456 
      patients from a single-center pulmonary ILD cohort whose diagnoses were 
      previously established by a multidisciplinary panel that did not include 
      rheumatologists. We reclassified patients as IPAF, CTD-ILD, or IPF. We compared 
      transplant-free survival using Kaplan-Meier methods and identified prognostic 
      factors using Cox models. RESULTS: We identified 60 patients with IPAF, 113 with 
      CTD-ILD, and 126 with IPF. Transplant-free survival of IPAF was not statistically 
      significantly different from that of CTD-ILD or IPF. Among IPAF patients, male 
      sex (hazard ratio, 4.58 [1.77-11.87]) was independently associated with worse 
      transplant-free survival. During follow-up, only 10% of IPAF patients were 
      diagnosed with CTD-ILD, most commonly antisynthetase syndrome. CONCLUSION: 
      Despite similar clinical characteristics, most patients with IPAF did not 
      progress to CTD-ILD; those who did often developed antisynthetase syndrome, 
      highlighting the critical importance of comprehensive myositis autoantibody 
      testing in this population. As in other types of ILD, male sex may portend a 
      worse prognosis in IPAF. The routine engagement of rheumatologists in the 
      multidisciplinary evaluation of ILD will help ensure the accurate classification 
      of these patients and help clarify prognostic factors.
CI  - Copyright (c) 2022 Wolters Kluwer Health, Inc. All rights reserved.
FAU - Jiwrajka, Nikhil
AU  - Jiwrajka N
AD  - From the Division of Rheumatology, Hospital of the University of Pennsylvania.
FAU - Loizidis, Giorgos
AU  - Loizidis G
AD  - Division of Rheumatology, Thomas Jefferson University Hospitals.
FAU - Patterson, Karen C
AU  - Patterson KC
FAU - Kreider, Maryl E
AU  - Kreider ME
AD  - Division of Pulmonary, Allergy, and Critical Care, Hospital of the University of 
      Pennsylvania, Philadelphia, PA.
FAU - Johnson, Cheilonda R
AU  - Johnson CR
AD  - Division of Pulmonary, Allergy, and Critical Care, Hospital of the University of 
      Pennsylvania, Philadelphia, PA.
FAU - Miller, Wallace T Jr
AU  - Miller WT Jr
AD  - Department of Radiology, Hospital of the University of Pennsylvania.
FAU - Barbosa, Eduardo Jose Mortani Jr
AU  - Barbosa EJM Jr
AD  - Department of Radiology, Hospital of the University of Pennsylvania.
FAU - Patel, Namrata
AU  - Patel N
AD  - Division of Pulmonary, Allergy, and Critical Care, Hospital of the University of 
      Pennsylvania, Philadelphia, PA.
FAU - Beers, Michael F
AU  - Beers MF
AD  - Division of Pulmonary, Allergy, and Critical Care, Hospital of the University of 
      Pennsylvania, Philadelphia, PA.
FAU - Litzky, Leslie A
AU  - Litzky LA
AD  - Department of Pathology and Laboratory Medicine, Hospital of the University of 
      Pennsylvania.
FAU - George, Michael D
AU  - George MD
FAU - Porteous, Mary K
AU  - Porteous MK
AD  - Division of Pulmonary, Allergy, and Critical Care, Hospital of the University of 
      Pennsylvania, Philadelphia, PA.
LA  - eng
PT  - Journal Article
DEP - 20220611
PL  - United States
TA  - J Clin Rheumatol
JT  - Journal of clinical rheumatology : practical reports on rheumatic & 
      musculoskeletal diseases
JID - 9518034
RN  - Antisynthetase syndrome
SB  - IM
MH  - *Autoimmune Diseases/complications/diagnosis
MH  - *Connective Tissue Diseases/complications/diagnosis
MH  - Humans
MH  - *Idiopathic Pulmonary Fibrosis/complications
MH  - *Lung Diseases, Interstitial/diagnosis
MH  - Male
MH  - *Myositis/complications/diagnosis
MH  - Prognosis
COIS- M.D.G. is supported by the National Institutes of Health Grant K23 AR073931-01 
      and has received consulting fees from AbbVie and research support from 
      Bristol-Myers Squibb and GlaxoSmithKline for unrelated work. M.F.B. is supported 
      by National Institutes of Health grant U01 HL152970, by The Robert L Mayock and 
      David A Cooper Endowed Professorship, and via unrestricted funds from the 
      Perelman School of Medicine Department of Medicine Research Fund. M.K.P. is 
      supported by The William "Dutch" Dyson Fund. The other authors declare no 
      conflict of interest.
EDAT- 2022/06/14 06:00
MHDA- 2022/07/28 06:00
CRDT- 2022/06/13 18:43
PHST- 2022/06/14 06:00 [pubmed]
PHST- 2022/07/28 06:00 [medline]
PHST- 2022/06/13 18:43 [entrez]
AID - 00124743-202208000-00005 [pii]
AID - 10.1097/RHU.0000000000001847 [doi]
PST - ppublish
SO  - J Clin Rheumatol. 2022 Aug 1;28(5):257-264. doi: 10.1097/RHU.0000000000001847. 
      Epub 2022 Jun 11.