Natural history of pulmonary fibrosis in two subjects with the same telomerase mutation

S El-Chemaly, SG Ziegler, RT Calado, KA Wilson… - Chest, 2011 - Elsevier
Previous studies have identified subclinical lung disease in family members of probands
with familial pulmonary fibrosis, but the natural history of preclinical pulmonary fibrosis is
uncertain. The purpose of this study was to determine whether individuals with preclinical
lung disease will develop pulmonary fibrosis. After a 27-year interval, two subjects with
manifestations of preclinical familial pulmonary fibrosis, including asymptomatic alveolar
inflammation and alveolar macrophage activation, were reevaluated for lung disease. CT …