CTICasesHermansky-Pudlak Syndrome Pulmonary Fibrosis with Asymmetric Cystic Pattern
Imaging findings
Another Puerto Rican patient with Hermansky-Pudlak syndrome had peripheral ground-glass opacity, reticulation, and unusual cystic spaces confirmed on surgical biopsy to represent fibrosis, with a notably asymmetric distribution and areas that resembled organizing pneumonia in places, without much additional clinical information available.
Key takeaways
Hermansky-Pudlak syndrome pulmonary fibrosis can present with an asymmetric, cystic pattern that superficially resembles organizing pneumonia in areas, further underscoring that familial/genetic fibrosing lung diseases as a group produce imaging patterns that are strange and asymmetric and cannot be forced into a standard idiopathic interstitial pneumonia differential (UIP versus NSIP), best described simply as fibrosis not otherwise specified pending genetic evaluation.
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