Idiopathic Pulmonary Fibrosis and Progressive Pulmonary Fibrosis in Adults

Publication Date: April 28, 2022

Introduction

Introduction

  • Progress in the diagnosis and treatment of idiopathic pulmonary fibrosis (IPF) and other interstitial lung diseases (ILDs) manifesting progressive pulmonary fibrosis (PPF) has generated the need for updating guidelines.
  • These recommendations are not mandates because they cannot account for all unique clinical circumstances and should be revisited as new evidence is published.

Recommendations

...mmendations

...on the Diagnosis and Treatment of IPF...

...transbronchial lung cryobiopsy (TBL...

We make no recommendation for or against...

...t not treating patients with IPF with...

...t not referring patients with IPF for anti-reflu...


...art II. Diagnosis and Treatment of...

...rfenidone...

...mmend further research into the efficacy...

...tedanib

...uggest nintedanib for the treatment of PPF in...

...commend research into the efficacy, e...


Tables

...bles

...High-resolution Computed Tomograph...


...le 2. Definition of PPFHaving trouble viewin...


...cted Fibrotic Lung Diseases That Can Manife...


Figures

Figures

...igure 1. IPF Diagnosis Based Upon HRCT and Biops...


...ure 2. Diagnostic Algorithm for IPF...


...chematic Pathway for Clinical Management of...


...D Manifesting PPFa The committee ack...


...action Bronchiectasis/bronchiolectasisAxi...


...HoneycombingAxial, sagittal, and coronal CT im...


...aseptal EmphysemaAxial and coronal CT images s...


...irspace Enlargement With Fibrosis (AEF...


...9. Spectrum of CT Appearances in UIP Patter...


...igure 10. UIP Pattern Due to Connective D...


...ure 11. Combined PPFE and UIP PatternsCoronal...


...gure 12. Three of the Four High-resolution CT...


...re 13. PPF in a Patient With IPF (Probable...


...ure 14. PPF Due to NSIPA) CT in a 45...