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
...mmendations...
...I. Update on the Diagnosis and Treatment of IPF...
...transbronchial lung cryobiopsy (TBL...
...recommendation for or against the addition of ge...
We suggest not treating patients with I...
...ggest not referring patients with IPF for anti...
.... Diagnosis and Treatment of PPF Other Than IPF...
...rfenidone...
...e recommend further research into the eff...
...ntedanib
...est nintedanib for the treatment of PPF in patie...
...recommend research into the efficacy, effectiven...
Tables
Tabl...
...e 1. High-resolution Computed Tomography (HRCT) P...
Table 2. Definition of PPFHaving t...
.... Selected Fibrotic Lung Diseases That...
Figures
...gures...
...1. IPF Diagnosis Based Upon HRCT and Bi...
...Diagnostic Algorithm for IPFa Patients with a...
...matic Pathway for Clinical Management...
...Manifesting PPFa The committee ackno...
...e 5. Traction Bronchiectasis/bronchiolectasisA...
.... HoneycombingAxial, sagittal, and coron...
...gure 7. Paraseptal EmphysemaAxial and coron...
.... Airspace Enlargement With Fibrosis (AEF), Also C...
...gure 9. Spectrum of CT Appearances in UIP P...
...e 10. UIP Pattern Due to Connective Disease C...
...Combined PPFE and UIP PatternsCoronal CT im...
...ee of the Four High-resolution CT Patterns...
...re 13. PPF in a Patient With IPF (Prob...
...PPF Due to NSIPA) CT in a 45 year old woman wit...