Familial idiopathic pulmonary fibrosis occurring in four members of a family

Abstract

Idiopathic pulmonary fibrosis (IPF) is a progressive fatal lung disease characterised by persistent pulmonary inflammation. Approximately 2% of cases are familial. Herein, we describe a family in which four members over two generations developed IPF. Of particular interest, the age of onset was lower in the second generation affected by the disease. Familial IPF offers a unique opportunity to study the proinflammatory processes implicated in IPF.