Kanj Nadim, Abdallah Mouhamad, Tawil Ayman
Department of Internal Medicine, American University of Beirut, Lebanon.
J Med Liban. 2006 Jan-Mar;54(1):45-9.
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.
