Familial Pulmonary Fibrosis

When two or more members within the same family have Idiopathic Pulmonary Fibrosis (IPF) or any other form of Idiopathic Interstitial Pneumonia (IIP), it is called Familial Pulmonary Fibrosis (FPF) or Familial Interstitial Pneumonia (FIP). The most common definition of FPF is two or more primary biological family members (parent, child, or sibling) with the diagnosis of IPF or IIP.  However, family members can be second degree as well, for example they can be an aunt or uncle, cousins, etc.

Moreover, FPF can occur with other types of IIP, such as non-specific interstitial pneumonia (NSIP), desquamative interstitial pneumonia (DIP), respiratory bronchiolitis associated interstitial lung disease (RB-ILD), and cryptogenic organizing pneumonia (COP). 

Learn more about FPF on our Familial Pulmonary Fibrosis site, developed by the Integrated Center for Genes, Environment, and Health.


This information has been approved by Kevin Brown, MD (January 2010).