CTICasesFamilial Pulmonary Fibrosis from Surfactant Protein C Deficiency with Incidental Lung Adenocarcinoma in the Affected Parent
Imaging findings
A 17-year-old with milder fine fibrosis and reticulation, more pronounced in the upper than lower lobes, was found on surgical pathology to have a mixed NSIP/UIP pattern with prominent bronchiolocentric fibrosis and was confirmed to have surfactant protein C deficiency. His father, who had presented earlier with reticulation and fine central fibrosis on thick-slice CT, was found on subsequent imaging to have a persistent and enlarging right lower lobe nodule that proved to be adenocarcinoma, resected via right lower lobectomy at age 42.
Key takeaways
Surfactant protein C deficiency can present with variable severity across family members and with a mixed, non-classifiable fibrotic pattern (often bronchiolocentric) on pathology, again illustrating why unclassifiable fibrosis warrants a search for a familial cause; the development of lung adenocarcinoma in a relatively young affected parent raises the possibility of increased lung cancer risk in the setting of fibrotic lung disease, though a specific association with surfactant protein C deficiency was not established in the literature.
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