Familial idiopathic pulmonary fibrosis in three Lebanese siblings. Case report with long-term follow-up.
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.
Kanj
Nadim
N
Department of Internal Medicine, American University of Beirut, Lebanon. dk01@aub.edu.lb
Abdallah
Mouhamad
M
Tawil
Ayman
A
D000328:Adult; D005192:Family Health; D005260:Female; D006801:Humans; D007861:Lebanon; D008297:Male; D011658:Pulmonary Fibrosis / Q000175:diagnosis / Q000235:genetics / Q000473:pathology