What is familial pulmonary fibrosis?

Familial pulmonary fibrosis is a term used when members of the same family develop idiopathic pulmonary fibrosis. To be precise, the definition is “at least one other first degree relative (parent, sib, or offspring) with idiopathic pulmonary fibrosis”

Familial pulmonary fibrosis has been studied, however the exact mechanism of inheritance is unclear. It is not known whether it is autosomal dominant or autosomal recessive. Three genes have however been implicated in the development of familial pulmonary fibrosis – TERTTERC, and SFTPC. 

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