PMID- 31681774
OWN - NLM
STAT- PubMed-not-MEDLINE
LR  - 20200930
IS  - 2296-858X (Print)
IS  - 2296-858X (Electronic)
IS  - 2296-858X (Linking)
VI  - 6
DP  - 2019
TI  - Interstitial Pneumonia With Autoimmune Features (IPAF).
PG  - 209
LID - 10.3389/fmed.2019.00209 [doi]
LID - 209
AB  - A significant proportion of patients with interstitial lung disease (ILD) 
      manifest autoimmune features, but do not fulfill the diagnostic criteria for a 
      definite connective tissue disease (CTD). In 2015, the European Respiratory 
      Society (ERS) and American Thoracic Society (ATS) "Task Force on undifferentiated 
      Forms of connective tissue disease-associated interstitial lung disease" proposed 
      classification criteria for a so-called research category of Interstitial 
      Pneumonia with Autoimmune Features (IPAF). These classification criteria were 
      based on a combination of features 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 multi-compartment involvement. Patients meeting IPAF criteria tend to 
      have a history of smoking similar to patients with idiopathic pulmonary fibrosis. 
      The most frequent clinical and serological markers of autoimmune features are 
      Raynaud' phenomenon and positive antinuclear antibodies, respectively. 
      Non-specific interstitial pneumonia is the predominant radiologic and 
      histopathologic pattern, although patients meeting IPAF criteria through the 
      clinical and serologic domains may also have a usual interstitial pneumonia 
      pattern. Management should be carefully individualized on a case-by-case basis in 
      keeping with the wide heterogeneity of IPAF and lack of evidence in this 
      particular subgroup of patients. Prognosis is generally intermediate between that 
      of idiopathic pulmonary fibrosis and connective tissue disease-associated 
      interstitial lung disease, but substantially variable according to the 
      predominant histologic and radiologic patterns. As acknowledged by the Task 
      Force, the proposed classification scheme of IPAF is a research concept that will 
      need revision and refinement based on data to better inform prognostication and 
      patient care.
CI  - Copyright (c) 2019 Fernandes, Nasser, Ahmad and Cottin.
FAU - Fernandes, Ligia
AU  - Fernandes L
AD  - Departamento do Torax, Centro Hospitalar Lisboa Norte, Lisbon, Portugal.
FAU - Nasser, Mouhamad
AU  - Nasser M
AD  - Department of Respiratory Medicine, National Reference Center for Rare Pulmonary 
      Diseases, Hospices Civils de Lyon, Lyon, France.
FAU - Ahmad, Kais
AU  - Ahmad K
AD  - Department of Respiratory Medicine, National Reference Center for Rare Pulmonary 
      Diseases, Hospices Civils de Lyon, Lyon, France.
FAU - Cottin, Vincent
AU  - Cottin V
AD  - Department of Respiratory Medicine, National Reference Center for Rare Pulmonary 
      Diseases, Hospices Civils de Lyon, Lyon, France.
AD  - Claude Bernard Lyon 1 University, University of Lyon, INRA, UMR754, Lyon, France.
LA  - eng
PT  - Journal Article
PT  - Review
DEP - 20190927
PL  - Switzerland
TA  - Front Med (Lausanne)
JT  - Frontiers in medicine
JID - 101648047
PMC - PMC6798044
OTO - NOTNLM
OT  - antibody
OT  - autoimmunity
OT  - classification
OT  - connective tissue disease
OT  - pulmonary fibrosis
EDAT- 2019/11/05 06:00
MHDA- 2019/11/05 06:01
PMCR- 2019/09/27
CRDT- 2019/11/05 06:00
PHST- 2019/07/31 00:00 [received]
PHST- 2019/09/09 00:00 [accepted]
PHST- 2019/11/05 06:00 [entrez]
PHST- 2019/11/05 06:00 [pubmed]
PHST- 2019/11/05 06:01 [medline]
PHST- 2019/09/27 00:00 [pmc-release]
AID - 10.3389/fmed.2019.00209 [doi]
PST - epublish
SO  - Front Med (Lausanne). 2019 Sep 27;6:209. doi: 10.3389/fmed.2019.00209. 
      eCollection 2019.