Familial Pulmonary Fibrosis

Reviewed by Jeffrey James Swigris, DO, MS

FPF family 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). 

Types of IIP in families

An estimated 10-15 percent of patients with idiopathic pulmonary fibrosis (IPF) have a form of the pulmonary fibrosis that runs in families.  This is called Familial Pulmonary Fibrosis (FPF).

The most common definition of Familial Pulmonary Fibrosis (FPF) is when two or more primary biological family members (parent, child, or sibling) have the diagnosis of an Idiopathic Interstitial Pneumonia (IIP) and at least one of the family members has the most common form of IIP, Idiopathic Pulmonary Fibrosis (IPF).  However, family members can be second degree as well.  For example they can be an aunt or uncle or cousins.

In about half of families with FPF, not all family members have the same type of IIP. 

In some families with FPF, not every affected family member has the same type of IIP.  In about half of families with FPF, one or more family members have idiopathic pulmonary fibrosis (IPF) and another has a different form of Idiopathic Interstitial Pneumonia.  Other forms could be: non-specific interstitial pneumonia (NSIP); desquamative interstitial pneumonia (DIP); respiratory bronchiolitis associated interstitial lung disease (RB-ILD); and cryptogenic organizing pneumonia (COP).

Cigarette smoking is a major risk factor for developing IIP, even in families with FPF.  

New had happen

For more than 100 years, National Jewish Health has been committed to finding new treatments and cures for diseases. Search our clinical trials.