Figure 1. Genetic variants define an inherited susceptibility to pulmonary fibrosis and to different manifestations of organ fibrosis.

Genes linked to an inherited risk of pulmonary fibrosis can be broadly classified into those that lead to a DNA damage response, those that are expressed in lung epithelium, and those that are expressed in lamellar bodies — or organelles crucial for type II alveolar epithelial cell function. Examples of other manifestations of organ fibrosis using this same broad classification scheme of variants affecting DNA, protein, and organelle function are indicated to the right. Genetic disorders characterized by the involvement of multiple different organs are listed with each panel.