Preclinical interstitial lung disease in relatives of familial pulmonary fibrosis patients

Familial pulmonary fibrosis: Defining inherited fibrotic lung disease in the era of clinical genetic testing

“…10 Of considerable recent interest are studies that sought to identify fPF prospectively by radiological screening of unaffected (by self-report) first-degree relatives of PF patients. Multiple studies have now provided evidence that more than a third of first-degree relatives of PF patients show early clinical signs of PF, [11][12][13][14] irrespective of whether the presenting IPF patient was classified as a familial or sporadic case. This has raised the concern that fPF is significantly under-diagnosed, with an associated missed opportunity for early intervention.…”