CTICasesFamilial Nonspecific Interstitial Pneumonia
Imaging findings
High-resolution chest CT scans in identical twin sisters demonstrate near-identical patterns of pulmonary fibrosis, characterized by uniform, peripheral, and subpleural lower-lobe-predominant ground-glass opacity, reticulation, traction bronchiectasis, and uniform cystic airspaces mimicking honeycombing. There is relative subpleural sparing.
Key takeaways
Familial pulmonary fibrosis can present with a nonspecific interstitial pneumonia (NSIP) pattern. When identical twins exhibit nearly identical patterns and distributions of fibrosing lung disease at a relatively young age (such as their 40s), a strong genetic predisposition is suspected (e.g., TERT or other telomerase-related mutations). This highlights the role of genetic factors in determining the specific fibrotic phenotype.
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