Most studies of telomere-related pulmonary fibrosis have focused on families in which one of several different telomere-associated ailments cosegregate with rare genetic mutations. Kindreds fall into two broad categories. First, for those with a diagnosis of dyskeratosis congenita, pulmonary fibrosis develops after the appearance of characteristic mucocutaneous features, usually following bone marrow failure during childhood or young adulthood.4 These patients have extremely short age-adjusted telomere lengths, usually less than the first percentile of normal. Second, patients with familial pulmonary fibrosis have lung disease as the dominant and presenting clinical feature, with an age of onset typically 50 years or older.5,6 For the latter group, hematologic abnormalities are mild or nonexistent and blood telomere lengths are not quite so short, usually within the lower 10th percentile. Although families with Mendelian single-gene telomeropathies have been well studied, prospective evaluations of cohorts of patients presenting with severe ILD and short telomere lengths are few.