To date, autosomal dominant mutations in four genes are known to cause adult onset IPF: SFTPC, SFTPA2, TERC and TERT. We screened these genes in our familial (n = 28) and sporadic (n = 50) probands, 95% of whom belong to the Newfoundland founder population. The gene discussed is SFTPC; the disease is idiopathic pulmonary fibrosis.