PMID- 35349416
OWN - NLM
STAT- MEDLINE
DCOM- 20220711
LR  - 20220711
IS  - 0392-856X (Print)
IS  - 0392-856X (Linking)
VI  - 40
IP  - 7
DP  - 2022 Jul
TI  - "Usual" interstitial pneumonia with autoimmune features: a prospective study on a 
      cohort of idiopathic pulmonary fibrosis patients.
PG  - 1324-1329
LID - 10.55563/clinexprheumatol/lqi6z7 [doi]
AB  - OBJECTIVES: The classification interstitial pneumonia with autoimmune features 
      (IPAF) includes patients with interstitial lung disease (ILD) associated with 
      autoimmune characteristics insufficient to reach classification criteria for a 
      specific autoimmune disease (SAD). These criteria are divided into three domains: 
      clinical, serological and morphological. The latter domain does not include the 
      usual interstitial pneumonia (UIP) pattern, which is deemed not to be 
      significantly associated with SAD. Therefore, the enrolment of these patients is 
      more difficult, requiring at least one item from both of the other domains. The 
      objective of this study is to evaluate the rate of progression towards SAD of a 
      cohort of UIP patients satisfying only one IPAF domain (we called this group 
      "UIPAF") compared with classic idiopathic pulmonary fibrosis (IPF). METHODS: We 
      prospectively enrolled IPF patients with radiologic and/or histologic UIP 
      pattern, followed jointly by rheumatologists and pulmonologists from January 2017 
      to January 2021, with a minimum follow-up of 12 months. RESULTS: We enrolled 190 
      IPF patients, 38 (20%) of whom were classified as UIPAF. IPF and UIPAF patients 
      were similar for general characteristics, severity and prognosis, at presentation 
      and at annual check-up. However, 28.9% of UIPAF patients progressed towards SAD, 
      compared with 2% of IPF patients (chi2=30.4, p</=0.0001). CONCLUSIONS: The 
      association between a single clinical or serological domain of IPAF and UIP 
      pattern is predictive for the development of a SAD if compared with isolated UIP. 
      ILD can be the first manifestation of SAD, even with a UIP pattern, therefore, 
      the morphological domain of IPAF criteria could be removed.
FAU - Sambataro, Gianluca
AU  - Sambataro G
AD  - Department of Clinical and Experimental Medicine, Regional Referral Centre for 
      Rare Lung Disease, A.O.U. Policlinico-San Marco, University of Catania, and 
      Outpatient Clinic of Rheumatology, Artroreuma S.r.l., Mascalucia (CT), Italy. 
      dottorsambataro@gmail.com.
FAU - Ferrara, Chiara A
AU  - Ferrara CA
AD  - Department of Clinical and Experimental Medicine, Regional Referral Centre for 
      Rare Lung Disease, A.O.U. Policlinico-San Marco, University of Catania, Italy.
FAU - Torrisi, Sebastiano E
AU  - Torrisi SE
AD  - Department of Clinical and Experimental Medicine, Regional Referral Centre for 
      Rare Lung Disease, A.O.U. Policlinico-San Marco, University of Catania, Italy.
FAU - Spadaro, Carla
AU  - Spadaro C
AD  - Department of Clinical and Experimental Medicine, Regional Referral Centre for 
      Rare Lung Disease, A.O.U. Policlinico-San Marco, University of Catania, Italy.
FAU - Vignigni, Giovanna
AU  - Vignigni G
AD  - Department of Clinical and Experimental Medicine, Regional Referral Centre for 
      Rare Lung Disease, A.O.U. Policlinico-San Marco, University of Catania, Italy.
FAU - Vancheri, Ada
AU  - Vancheri A
AD  - Department of Clinical and Experimental Medicine, Regional Referral Centre for 
      Rare Lung Disease, A.O.U. Policlinico-San Marco, University of Catania, Italy.
FAU - Del Papa, Nicoletta
AU  - Del Papa N
AD  - Day Hospital of Rheumatology, Department of Rheumatology, ASST G.Pini-CTO, Milan, 
      Italy.
FAU - Orlandi, Martina
AU  - Orlandi M
AD  - Department of Experimental and Clinical Biomedical Sciences, Radiodiagnostic Unit 
      2, University of Florence - AOUC, Florence, Italy.
FAU - Colaci, Michele
AU  - Colaci M
AD  - Department of Clinical and Experimental Medicine, Internal Medicine Unit, 
      Cannizzaro Hospital, University of Catania, Italy.
FAU - Malatino, Lorenzo
AU  - Malatino L
AD  - Department of Clinical and Experimental Medicine, Internal Medicine Unit, 
      Cannizzaro Hospital, University of Catania, Italy.
FAU - Palmucci, Stefano
AU  - Palmucci S
AD  - Department of Medical, Surgical Sciences and Advanced Technologies, G.F. 
      Ingrassia, University of Catania, Italy.
FAU - Cavagna, Lorenzo
AU  - Cavagna L
AD  - Division of Rheumatology, University and IRCCS Policlinico S. Matteo, Pavia, 
      Italy.
FAU - Sambataro, Domenico
AU  - Sambataro D
AD  - Outpatient Clinic of Rheumatology, Artroreuma S.r.l., Mascalucia (CT), and 
      5Department of Clinical and Experimental Medicine, Internal Medicine Unit, 
      Cannizzaro Hospital, University of Catania, Italy.
FAU - Vancheri, Carlo
AU  - Vancheri C
AD  - Department of Clinical and Experimental Medicine, Regional Referral Centre for 
      Rare Lung Disease, A.O.U. Policlinico-San Marco, University of Catania, Italy.
LA  - eng
PT  - Journal Article
DEP - 20220309
PL  - Italy
TA  - Clin Exp Rheumatol
JT  - Clinical and experimental rheumatology
JID - 8308521
SB  - IM
MH  - *Autoimmune Diseases/complications/diagnostic imaging
MH  - Humans
MH  - *Idiopathic Pulmonary Fibrosis/diagnosis/diagnostic imaging
MH  - *Lung Diseases, Interstitial/diagnostic imaging/etiology
MH  - Prospective Studies
MH  - Retrospective Studies
MH  - Tomography, X-Ray Computed
EDAT- 2022/03/30 06:00
MHDA- 2022/07/12 06:00
CRDT- 2022/03/29 17:12
PHST- 2021/05/15 00:00 [received]
PHST- 2021/07/09 00:00 [accepted]
PHST- 2022/03/30 06:00 [pubmed]
PHST- 2022/07/12 06:00 [medline]
PHST- 2022/03/29 17:12 [entrez]
AID - 17427 [pii]
AID - 10.55563/clinexprheumatol/lqi6z7 [doi]
PST - ppublish
SO  - Clin Exp Rheumatol. 2022 Jul;40(7):1324-1329. doi: 
      10.55563/clinexprheumatol/lqi6z7. Epub 2022 Mar 9.