PMID- 33375368
OWN - NLM
STAT- PubMed-not-MEDLINE
LR  - 20210218
IS  - 2227-9059 (Print)
IS  - 2227-9059 (Electronic)
IS  - 2227-9059 (Linking)
VI  - 9
IP  - 1
DP  - 2020 Dec 26
TI  - Interstitial Pneumonia with Autoimmune Features: Why Rheumatologist-Pulmonologist 
      Collaboration Is Essential.
LID - 10.3390/biomedicines9010017 [doi]
LID - 17
AB  - In 2015 the European Respiratory Society (ERS) and the American Thoracic Society 
      (ATS) "Task Force on Undifferentiated Forms of Connective Tissue 
      Disease-associated Interstitial Lung Disease" proposed classification criteria 
      for a new research category defined as "Interstitial Pneumonia with Autoimmune 
      Features" (IPAF), to uniformly define patients with interstitial lung disease 
      (ILD) and features of autoimmunity, without a definite connective tissue disease. 
      These classification criteria were based on a variable combination of features 
      obtained from three domains: a clinical domain consisting of extra-thoracic 
      features, a serologic domain with specific autoantibodies, and a morphologic 
      domain with imaging patterns, histopathological findings, or multicompartment 
      involvement. Features suggesting a systemic vasculitis were excluded. Since 
      publication of ERS/ATS IPAF research criteria, various retrospective studies have 
      been published focusing on prevalence; clinical, morphological, and serological 
      features; and prognosis of these patients showing a broad heterogeneity in the 
      results. Recently, two prospective, cohort studies were performed, confirming the 
      existence of some peculiarities for this clinical entity and the possible 
      progression of IPAF to a defined connective tissue disease (CTD) in about 15% of 
      cases. Moreover, a non-specific interstitial pneumonia pattern, an anti-nuclear 
      antibody positivity, and a Raynaud phenomenon were the most common findings. In 
      comparison with idiopathic pulmonary fibrosis (IPF), IPAF patients showed a 
      better performance in pulmonary function tests and less necessity of oxygen 
      delivery. However, at this stage of our knowledge, we believe that further 
      prospective studies, possibly derived from multicenter cohorts and through 
      randomized control trials, to further validate the proposed classification 
      criteria are needed.
FAU - Sebastiani, Marco
AU  - Sebastiani M
AUID- ORCID: 0000-0002-1294-6421
AD  - Rheumatology Unit, University of Modena and Reggio Emilia, Azienda 
      Ospedaliero-Universitaria Policlinico di Modena, 41124 Modena, Italy.
FAU - Faverio, Paola
AU  - Faverio P
AD  - Respiratory Unit, San Gerardo Hospital, Department of Medicine and Surgery, 
      University of Milan-Bicocca, 20900 Monza, Italy.
FAU - Manfredi, Andreina
AU  - Manfredi A
AD  - Rheumatology Unit, University of Modena and Reggio Emilia, Azienda 
      Ospedaliero-Universitaria Policlinico di Modena, 41124 Modena, Italy.
FAU - Cassone, Giulia
AU  - Cassone G
AUID- ORCID: 0000-0002-3454-401X
AD  - Rheumatology Unit, University of Modena and Reggio Emilia, Azienda 
      Ospedaliero-Universitaria Policlinico di Modena, 41124 Modena, Italy.
FAU - Vacchi, Caterina
AU  - Vacchi C
AD  - Rheumatology Unit, University of Modena and Reggio Emilia, Azienda 
      Ospedaliero-Universitaria Policlinico di Modena, 41124 Modena, Italy.
FAU - Stainer, Anna
AU  - Stainer A
AD  - Respiratory Unit, San Gerardo Hospital, Department of Medicine and Surgery, 
      University of Milan-Bicocca, 20900 Monza, Italy.
FAU - Pozzi, Maria Rosa
AU  - Pozzi MR
AD  - Rheumatology Unit, San Gerardo Hospital, 20900 Monza, Italy.
FAU - Salvarani, Carlo
AU  - Salvarani C
AD  - Rheumatology Unit, University of Modena and Reggio Emilia, Azienda 
      Ospedaliero-Universitaria Policlinico di Modena, 41124 Modena, Italy.
AD  - Rheumatology Unit, Dipartimento Medicina Interna e Specialita Mediche, Azienda 
      Unita Sanitaria Locale di Reggio Emilia-Istituto di Ricerca e Cura a Carattere 
      Scientifico, 42123 Reggio Emilia, Italy.
FAU - Pesci, Alberto
AU  - Pesci A
AD  - Respiratory Unit, San Gerardo Hospital, Department of Medicine and Surgery, 
      University of Milan-Bicocca, 20900 Monza, Italy.
FAU - Luppi, Fabrizio
AU  - Luppi F
AUID- ORCID: 0000-0001-5775-5947
AD  - Respiratory Unit, San Gerardo Hospital, Department of Medicine and Surgery, 
      University of Milan-Bicocca, 20900 Monza, Italy.
LA  - eng
PT  - Journal Article
PT  - Review
DEP - 20201226
PL  - Switzerland
TA  - Biomedicines
JT  - Biomedicines
JID - 101691304
PMC - PMC7824155
OTO - NOTNLM
OT  - antibody
OT  - autoimmunity
OT  - classification
OT  - connective tissue diseases
OT  - idiopathic interstitial pneumonias
OT  - interstitial lung diseases
OT  - interstitial pneumonia with autoimmune features
OT  - prognosis
COIS- The authors declare no conflict of interest.
EDAT- 2020/12/31 06:00
MHDA- 2020/12/31 06:01
PMCR- 2020/12/26
CRDT- 2020/12/30 01:04
PHST- 2020/11/13 00:00 [received]
PHST- 2020/12/21 00:00 [revised]
PHST- 2020/12/24 00:00 [accepted]
PHST- 2020/12/30 01:04 [entrez]
PHST- 2020/12/31 06:00 [pubmed]
PHST- 2020/12/31 06:01 [medline]
PHST- 2020/12/26 00:00 [pmc-release]
AID - biomedicines9010017 [pii]
AID - biomedicines-09-00017 [pii]
AID - 10.3390/biomedicines9010017 [doi]
PST - epublish
SO  - Biomedicines. 2020 Dec 26;9(1):17. doi: 10.3390/biomedicines9010017.