Figure 2.

(A) Post-transplant survival according to presence or absence of telomere dysfunction. Patients with telomere dysfunction had a greater post-transplant follow up period due to an earlier requirement of lung transplant after pulmonary fibrosis diagnosis. As previously described in Table 2, a shorter time from diagnosis to transplantation was reported for patients with telomere dysfunction. (B) Survival after lung transplant according to family aggregation. A worse post-transplant survival was reported for family aggregation patients group during the first year. However, at 24 months, post-transplant survival was ≤ 50% regardless of family aggregation. Since genetic studies have not yet been implemented in routine clinical practice in most centers, further studies are needed to evaluate family aggregation as an outcome predictor regardless of the underlying genetic defect.