Photo Credit: DouglasOlivares
The following is a summary of “Progressive Early Interstitial Lung Abnormalities in Persons at Risk for Familial Pulmonary Fibrosis: A Prospective Cohort Study,” published in the December 2024 issue of Pulmonology by Salisbury et al.
Relatives of individuals with familial pulmonary fibrosis (FPF) were at increased risk of developing FPF, and the implications of very mild interstitial lung abnormalities (ILAs) as a radiologic biomarker for subclinical disease remained unclear.
Researchers conducted a retrospective study to quantify the progression risk among FPF relatives with abnormalities below the Fleischner Society’s ILAs threshold and to describe the characteristics of participants with new or progressive ILAs during observation.
They performed serial high-resolution chest computed tomography screening for asymptomatic FPF relatives. Early ILAs (no minimum threshold of lung involvement) were classified as mild (interstitial abnormalities involving <5% of a lung zone) or moderate (involvement of >5%). Progression was defined as the appearance of new or progressive ILAs on high-resolution chest computed tomography or the development of pulmonologist-diagnosed clinical FPF. Covariate-adjusted logistic regression identified characteristics associated with progression.
The results showed that among 273 participants (mean age 53.2 ± 9.4 years, 35% men, 27% ever-smokers), progression occurred during a mean follow-up of 6.2 ± 3.0 years in 15% of those without ILAs at enrollment, 65% with mild ILAs, and 77% with moderate ILAs. Mild ILAs were associated with 9.15 times (95% CI, 4.40–19.00; P < 0.0001) higher odds of progression, while moderate ILAs had 17.14 times (95% CI, 4.42–66.49; P < 0.0001) higher odds of progression compared to no ILAs.
Investigators concluded that in individuals at risk for FPF, minor interstitial abnormalities, such as unilateral reticulation or involvement of less than 5% of a lung zone, often signify subclinical disease.
Source: pubmed.ncbi.nlm.nih.gov/39137317