Role of Genetics in Idiopathic Pulmonary Fibrosis (IPF)

Familial Pulmonary Fibrosis (FPF) is a sub-category of the idiopathic interstitial pneumonias
(IIPs). IIPs are progressive lung conditions, with limited treatment options and unknown
etiology. Though the IIPs have been associated with both genetic risk factors and
environmental exposures, the molecular mechanism underlying disease progression remain poorly
understood. This investigation seeks to identify a group of genetic loci that play a role in
the development of familial interstitial pneumonia (FIP) or FPF, where 2 or more cases of IIP
are seen within a family.

Inclusion Criteria:

- Two or more family members with a clinical diagnosis of Idiopathic Pulmonary Fibrosis
(IPF) or Idiopathic Interstitial Pneumonia (IIP)

- Additional family members may be eligible to participate if two family members are
suspected of or diagnosed as having Idiopathic Pulmonary Fibrosis (IPF) or Idiopathic
Interstitial Pneumonia (IIP)

Exclusion Criteria:

- Individuals whose pulmonary fibrosis is due to a known cause rather than idiopathic

- Individuals whose pulmonary fibrosis is due to a broader genetic syndrome