Idiopathic Pulmonary Fibrosis and Progressive Pulmonary Fibrosis in Adults

Publication Date: April 29, 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

...ommendations...

...e on the Diagnosis and Treatment of I...

...suggest transbronchial lung cryobiopsy (...

We make no recommendation for or against...

...ot treating patients with IPF with antacid medic...

...e suggest not referring patients with IPF for...


Part II. Diagnosis and Treatment of PPF Other T...

...irfenidone...

...d further research into the efficac...

...tedanib...

...nintedanib for the treatment of PPF in patients w...

...nd research into the efficacy, effectiven...


Tables

Table...

...able 1. High-resolution Computed Tomography...


...ion of PPFHaving trouble viewing table? Exp...


...d Fibrotic Lung Diseases That Can Man...


Figures

...gures

...e 1. IPF Diagnosis Based Upon HRCT and...


.... Diagnostic Algorithm for IPFa Patients w...


...gure 3. Schematic Pathway for Clinical M...


...anifesting PPFa The committee acknowledges tha...


...e 5. Traction Bronchiectasis/bronchiolectasisA...


...gure 6. HoneycombingAxial, sagittal, and corona...


...7. Paraseptal EmphysemaAxial and coronal CT images...


...pace Enlargement With Fibrosis (AEF), Als...


...trum of CT Appearances in UIP Pattern Due to HP...


.... UIP Pattern Due to Connective Disease CTD-UIP P...


...Combined PPFE and UIP PatternsCorona...


...ure 12. Three of the Four High-resolut...


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


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