title: Familial idiopathic 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.
authors.affiliation: Department of Internal Medicine, American University of Beirut, Lebanon. dk01@aub.edu.lb
authors.forename: Nadim
authors.initials: N
authors.lastname: Kanj
authors.forename: Mouhamad
authors.initials: M
authors.lastname: Abdallah
authors.forename: Ayman
authors.initials: A
authors.lastname: Tawil
mesh_terms: D000328:Adult; D005192:Family Health; D005260:Female; D006801:Humans; D007861:Lebanon; D008297:Male; D011658:Pulmonary Fibrosis / Q000175:diagnosis / Q000235:genetics / Q000473:pathology