Taking the "I" out of IPF.

As the authors describe, the initial evidence that pulmonary fibrosis may have a genetic basis emerged from case reports and case series that reported multiple cases of idiopathic interstitial pneumonias (IIPs) clustering in families [2–6]. The epidemiology indicates that within families containing two or more cases of IIP (familial interstitial pneumonia) the development of familial interstitial pneumonia is associated with older age, male sex and ever having smoked cigarettes [6, 7]. The transmission pattern of inheritance is consistent with an autosomal dominant pattern, and 45% of the families demonstrated several subtypes of IIP within the same family, suggesting that IIP subtypes may be aetiologically related [6].