Fig. 1.

Telomere length in lymphocytes from IIP patients and families with known telomerase mutations compared with healthy controls. (A) IIP patients, in yellow, have shorter telomeres than age-matched controls (P < 0.0001, Wilcoxon signed rank). IIP patients [60 of 62 (97%)] have telomeres shorter than the median of healthy controls (P < 0.0001). Of 62 IIP patients, 50 (81%) carried the diagnosis of IPF. (B) Detailed view of A with individuals with features of a telomere syndrome highlighted in red: *, a 77-year-old IPF patient with hTR 325G→T mutation; §, a patient with very short telomeres who had chronic unexplained thrombocytopenia, a feature of subclinical aplastic anemia; ‡, two individuals with both IPF and cryptogenic liver cirrhosis who have short telomeres. Ten percent of IIP patients (6 of 62) have short telomeres below the first percentile; a range predictive of the presence of a telomerase mutation. (C) Telomere length from 45 individuals from 10 families with known mutations in hTERT (n = 17), hTR (n = 3), and DKC1 (n = 4). (D) Bar graph illustrates the mean difference in telomere length from the median of age-matched healthy controls. Compared with noncarriers whose telomere length was similar to controls (P = 0.304, Wilcoxon signed rank), both sporadic IIP patients and known telomerase mutation carriers had shorter telomeres (P < 0.0001 for both).