Definitions

• A term encompassing a heterogeneous group of disorders characterized by inflammation and progressive fibrosis of the lungs.

• Cough and progressive exertional dyspnea are the most common symptoms.

(IIPs): a group of interstitial lung diseases of unknown cause, also described as diffuse parenchymal lung diseases (DPLDs), characterized by inflammation and fibrosis of the pulmonary interstitium but frequently also affect the airspaces, peripheral airways, and vessels

Idiopathic interstitial pneumonias (IIPs)

• Idiopathic pulmonary fibrosis (IPF)

• Other IIPs

  • Acute interstitial pneumonia (AIP)

  • Cryptogenic organizing pneumonia (COP)

  • Respiratory bronchiolitis-associated interstitial lung disease (RB-ILD)

  • Desquamative interstitial pneumonia (DIP)

  • Nonspecific interstitial pneumonia (NSIP)

  • Lymphoid interstitial pneumonia (LIP)

  • Idiopathic pleuroparenchymal fibroelastosis