Familial idopathic pulmonary fibrosis in three lebanese siblings. Case report with long-term follow-up

ABSTRACT

This is the first report of a familial cluster of idiopathic pulmonary fibrosis [IPF] in Lebanon. This rare variant of IPF has an autosomal dominant mode of inheritance with variable expressivity, and is commonly associated with a mutation of the surfactant protein C gene. The patients are younger at diagnosis but have otherwise identical clinical, radiological, and histological features as the more common non-familial cases. IPF is an invariably fatal disease with no effective treatment. Lung transplantation remains the only chance for more prolonged survival and must be considered in young patients