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Related Concept Videos

Pneumonia I: Introduction01:30

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Interstitial Pneumonia With Autoimmune Features (IPAF).

Ligia Fernandes1, Mouhamad Nasser2, Kais Ahmad2

  • 1Departamento do Tórax, Centro Hospitalar Lisboa Norte, Lisbon, Portugal.

Frontiers in Medicine
|November 5, 2019
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Summary
This summary is machine-generated.

Interstitial Pneumonia with Autoimmune Features (IPAF) describes lung disease patients with autoimmune signs but not a defined connective tissue disease. IPAF criteria aid research but require refinement for patient care and prognosis.

Keywords:
antibodyautoimmunityclassificationconnective tissue diseasepulmonary fibrosis

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Area of Science:

  • Pulmonology
  • Rheumatology
  • Immunology

Background:

  • Many interstitial lung disease (ILD) patients show autoimmune features without meeting criteria for connective tissue disease (CTD).
  • The European Respiratory Society (ERS) and American Thoracic Society (ATS) proposed Interstitial Pneumonia with Autoimmune Features (IPAF) as a research category in 2015.
  • IPAF classification integrates clinical, serologic, and morphologic domains.

Purpose of the Study:

  • To define and classify interstitial lung disease with autoimmune features (IPAF).
  • To establish research criteria for IPAF, bridging ILD and CTD.
  • To guide further research and potential clinical management strategies for IPAF patients.

Main Methods:

  • Utilized a multi-domain approach: clinical (extra-thoracic features), serologic (autoantibodies), and morphologic (imaging, histology).
  • Identified frequent markers: Raynaud's phenomenon and antinuclear antibodies.
  • Compared IPAF patient characteristics, including smoking history and patterns, to idiopathic pulmonary fibrosis (IPF) and CTD-ILD.

Main Results:

  • IPAF patients share smoking history similarities with IPF.
  • Non-specific interstitial pneumonia (NSIP) is the predominant pattern, though usual interstitial pneumonia (UIP) can occur.
  • Prognosis is variable, generally intermediate between IPF and CTD-ILD.

Conclusions:

  • IPAF serves as a crucial research concept for patients with ILD and autoimmune features.
  • Individualized management is essential due to IPAF's heterogeneity and limited evidence.
  • Further data collection and refinement of IPAF criteria are needed for improved prognostication and patient care.