Idiopathic Pulmonary Fibrosis and Progressive Pulmonary Fibrosis in Adults
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
...mendations
...n the Diagnosis and Treatment of IPF...
...nsbronchial lung cryobiopsy (TBLC) be regard...
...ke no recommendation for or against the addi...
...reating patients with IPF with antacid medication...
We suggest not referring patients with IPF for a...
...Diagnosis and Treatment of PPF Other Than IP...
Pirfenido...
...further research into the efficacy, ef...
...tedanib...
...nintedanib for the treatment of PPF in patients...
...mend research into the efficacy, effectiven...
Tables
Tabl...
...High-resolution Computed Tomography (HRCT) Patter...
...Definition of PPFHaving trouble vie...
...elected Fibrotic Lung Diseases That...
Figures
...gures
...agnosis Based Upon HRCT and Biopsy P...
...iagnostic Algorithm for IPFa Patients with a r...
...gure 3. Schematic Pathway for Clinical...
...re 4. ILD Manifesting PPFa The commi...
...ure 5. Traction Bronchiectasis/bronchio...
...6. HoneycombingAxial, sagittal, and coronal CT...
Figure 7. Paraseptal EmphysemaAxial an...
.... Airspace Enlargement With Fibrosis (AE...
...gure 9. Spectrum of CT Appearances i...
...IP Pattern Due to Connective Disease CTD-...
...ombined PPFE and UIP PatternsCoronal CT imag...
...e 12. Three of the Four High-resolut...
...igure 13. PPF in a Patient With IPF...
...PPF Due to NSIPA) CT in a 45 year old woman with...