CTICasesFamilial Pulmonary Fibrosis
Imaging findings
Serial chest CT scans in a 57-year-old woman with a strong family history of interstitial lung disease (three siblings affected) show progressive, asymmetric pulmonary fibrosis. The pattern is non-classifiable, demonstrating traction bronchiectasis, volume loss, and focal areas of air trapping/mosaic attenuation in both lung bases, without classic subpleural honeycombing of a usual interstitial pneumonia (UIP) pattern. Explanted lung pathology confirms end-stage fibrosis.
Key takeaways
Familial pulmonary fibrosis (FPF) refers to idiopathic interstitial pneumonia occurring in two or more primary relatives. FPF can exhibit variable clinical courses and diverse histological patterns (including UIP, NSIP, or unclassifiable fibrosis) within the same family. FPF patients often present at a younger age than sporadic IPF cases, and genetic counseling/testing (e.g., for telomerase mutations) is indicated.
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