PMID- 38256503
OWN - NLM
STAT- PubMed-not-MEDLINE
LR  - 20240128
IS  - 2077-0383 (Print)
IS  - 2077-0383 (Electronic)
IS  - 2077-0383 (Linking)
VI  - 13
IP  - 2
DP  - 2024 Jan 10
TI  - The Pattern and Progression of "Usual" Interstitial Pneumonia with Autoimmune 
      Features: Comparison with Patients with Classic Interstitial Pneumonia with 
      Autoimmune Features and Idiopathic Pulmonary Fibrosis.
LID - 10.3390/jcm13020369 [doi]
LID - 369
AB  - BACKGROUND: We proposed the term "UIPAF" to define patients with Usual 
      Interstitial Pneumonia (UIP) associated with only one domain of the 
      classification called "Interstitial Pneumonia with Autoimmune Features" (IPAF). 
      The objective of this study was to evaluate the clinical presentation and 
      prognosis of UIPAF patients, compared with two cohorts, composed of IPAF and 
      idiopathic pulmonary fibrosis (IPF) patients, respectively. METHODS: The patients 
      were enrolled as IPAF, UIPAF, or IPF based on clinical, serological, and 
      radiological data and evaluated by a multidisciplinary team. RESULTS: We enrolled 
      110 patients with IPF, 69 UIPAF, and 123 IPAF subjects. UIPAF patients were 
      similar to IPAF regarding autoimmune features, except for the prevalence of 
      Rheumatoid Factor in UIPAF and anti-SSA in IPAF. A similar proportion of the two 
      cohorts progressed toward a specific autoimmune disease (SAD), with differences 
      in the kind of SAD developed. The real-life management and prognosis of UIPAF 
      patients proved to be almost identical to IPF. CONCLUSIONS: UIPAF shared with 
      IPAF similar autoimmune features, suggesting the opportunity to be considered 
      IPAF, excluding the morphological domain by the classification. However, the 
      real-life management and prognosis of UIPAF are similar to IPF. These data 
      suggest a possible modification in the therapeutic management of UIPAF.
FAU - Libra, Alessandro
AU  - Libra A
AD  - Department of Clinical and Experimental Medicine, Regional Referral Center for 
      Rare Lung Disease, Policlinico "G. Rodolico-San Marco", University of Catania, 
      95123 Catania, Italy.
FAU - Colaci, Michele
AU  - Colaci M
AUID- ORCID: 0000-0002-3606-3404
AD  - Internal Medicine Unit, Department of Clinical and Experimental Medicine, 
      Division of Rheumatology, Cannizzaro Hospital, University of Catania, 95123 
      Catania, Italy.
FAU - Spicuzza, Lucia
AU  - Spicuzza L
AUID- ORCID: 0000-0002-8246-8357
AD  - Department of Clinical and Experimental Medicine, Regional Referral Center for 
      Rare Lung Disease, Policlinico "G. Rodolico-San Marco", University of Catania, 
      95123 Catania, Italy.
FAU - Luca, Giuliana
AU  - Luca G
AUID- ORCID: 0009-0006-0324-9989
AD  - Department of Clinical and Experimental Medicine, Regional Referral Center for 
      Rare Lung Disease, Policlinico "G. Rodolico-San Marco", University of Catania, 
      95123 Catania, Italy.
FAU - Fischetti, Sefora
AU  - Fischetti S
AD  - Department of Clinical and Experimental Medicine, Regional Referral Center for 
      Rare Lung Disease, Policlinico "G. Rodolico-San Marco", University of Catania, 
      95123 Catania, Italy.
FAU - Pashalidis, Giorgio
AU  - Pashalidis G
AD  - Department of Clinical and Experimental Medicine, Regional Referral Center for 
      Rare Lung Disease, Policlinico "G. Rodolico-San Marco", University of Catania, 
      95123 Catania, Italy.
FAU - Ferrara, Chiara Alfia
AU  - Ferrara CA
AD  - Department of Clinical and Experimental Medicine, Regional Referral Center for 
      Rare Lung Disease, Policlinico "G. Rodolico-San Marco", University of Catania, 
      95123 Catania, Italy.
FAU - Ielo, Giuseppe
AU  - Ielo G
AD  - Department of Clinical and Experimental Medicine, Regional Referral Center for 
      Rare Lung Disease, Policlinico "G. Rodolico-San Marco", University of Catania, 
      95123 Catania, Italy.
FAU - Sambataro, Domenico
AU  - Sambataro D
AD  - Artroreuma s.r.l., Rheumatology Outpatient Clinic, 95030 Mascalucia (CT), Italy.
FAU - La Rosa, Giuliana
AU  - La Rosa G
AD  - Department of Medical Surgical Sciences and Advanced Technologies "GF Ingrassia", 
      University Hospital Policlinico "G. Rodolico-San Marco", 95123 Catania, Italy.
FAU - Libra, Federica
AU  - Libra F
AD  - Department of Medical Surgical Sciences and Advanced Technologies "GF Ingrassia", 
      University Hospital Policlinico "G. Rodolico-San Marco", 95123 Catania, Italy.
FAU - Palmucci, Stefano
AU  - Palmucci S
AUID- ORCID: 0000-0002-7196-2974
AD  - Department of Medical Surgical Sciences and Advanced Technologies "GF Ingrassia", 
      University Hospital Policlinico "G. Rodolico-San Marco", Unita Operativa Semplice 
      Dipartimentale di Imaging Polmonare e Tecniche Radiologiche Avanzate (UOSD 
      IPTRA), 95123 Catania, Italy.
FAU - Vancheri, Carlo
AU  - Vancheri C
AD  - Department of Clinical and Experimental Medicine, Regional Referral Center for 
      Rare Lung Disease, Policlinico "G. Rodolico-San Marco", University of Catania, 
      95123 Catania, Italy.
FAU - Sambataro, Gianluca
AU  - Sambataro G
AUID- ORCID: 0000-0001-9933-1202
AD  - Department of Clinical and Experimental Medicine, Regional Referral Center for 
      Rare Lung Disease, Policlinico "G. Rodolico-San Marco", University of Catania, 
      95123 Catania, Italy.
AD  - Internal Medicine Unit, Department of Clinical and Experimental Medicine, 
      Division of Rheumatology, Cannizzaro Hospital, University of Catania, 95123 
      Catania, Italy.
AD  - Artroreuma s.r.l., Rheumatology Outpatient Clinic, 95030 Mascalucia (CT), Italy.
LA  - eng
PT  - Journal Article
DEP - 20240110
PL  - Switzerland
TA  - J Clin Med
JT  - Journal of clinical medicine
JID - 101606588
PMC - PMC10816405
OTO - NOTNLM
OT  - connective tissue disease
OT  - diagnosis
OT  - idiopathic pulmonary fibrosis
OT  - interstitial pneumonia with autoimmune features
OT  - usual interstitial pneumonia
COIS- G.S. reports personal fees from Boehringer Ingelheim outside the submitted work; 
      S.P. reports personal fees from Boehringer Ingelheim, Delphi International srl, 
      and F. Hoffmann-La Roche Ltd. outside the submitted work; C.V. is part of the F. 
      Hoffmann-La Roche Ltd. and Boehringer Ingelheim Scientific board. He received 
      consulting fees and/or speaker fees from AstraZeneca, Boehringer Ingelheim, C.F. 
      Hoffmann-La Roche Ltd., and Menarini. C.A.F., D.S., L.S., G.L., G.P., S.F., M.C., 
      A.L., G.I., F.L. and G.L.R. declare no conflicts of interest.
EDAT- 2024/01/23 06:43
MHDA- 2024/01/23 06:44
PMCR- 2024/01/10
CRDT- 2024/01/23 01:15
PHST- 2023/12/20 00:00 [received]
PHST- 2024/01/06 00:00 [revised]
PHST- 2024/01/08 00:00 [accepted]
PHST- 2024/01/23 06:44 [medline]
PHST- 2024/01/23 06:43 [pubmed]
PHST- 2024/01/23 01:15 [entrez]
PHST- 2024/01/10 00:00 [pmc-release]
AID - jcm13020369 [pii]
AID - jcm-13-00369 [pii]
AID - 10.3390/jcm13020369 [doi]
PST - epublish
SO  - J Clin Med. 2024 Jan 10;13(2):369. doi: 10.3390/jcm13020369.