CTICasesHermansky-Pudlak Syndrome Pulmonary Fibrosis (Puerto Rican Patient, NSIP-like Pattern)
Imaging findings
A patient from Puerto Rico with albinism and other features of Hermansky-Pudlak syndrome had an old radiograph showing distortion and probable pulmonary hypertension; CT showed ground glass, reticulation, and traction bronchiectasis in a peribronchovascular distribution more reminiscent of NSIP than UIP, with a cyst noted and relative sparing of the lower lobes, again not fitting a typical UIP or hypersensitivity pneumonitis pattern.
Key takeaways
Hermansky-Pudlak-associated pulmonary fibrosis, historically well described in a specific founder population in Puerto Rico, can show an NSIP-like peribronchovascular pattern rather than classic UIP, reinforcing that these genetic fibrosing lung diseases frequently defy standard idiopathic interstitial pneumonia classification and should prompt consideration of the associated albinism/platelet phenotype.
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