Using Families to Study Pulmonary Fibrosis Genetics

Pulmonary fibrosis is typically classified as "sporadic," meaning there is no known family history of the condition, or "familial," meaning two or more related family members are affected. Recent research suggests that about one-fifth of instances of ILD and one-fourth of cases of idiopathic pulmonary fibrosis (IPF) are familial. People with familial pulmonary fibrosis, whether they have IPF or not, are more likely to have the disease in a progressive form. It is generally accepted that pulmonary fibrosis develops in those who have a genetic predisposition to the condition and are then exposed to an environmental factor. Pulmonary fibrosis, however, seems to be inherited in a far more Mendelian manner in some families.