Journal
INTERNAL MEDICINE
Volume 60, Issue 5, Pages 783-788Publisher
JAPAN SOC INTERNAL MEDICINE
DOI: 10.2169/internalmedicine.5493-20
Keywords
interstitial pneumonia; Herrnansky-Pudlak syndrome type 4; idiopathic pulmonary fibrosis; antifibrotic agents; nintedanib; pirfenidone
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Hermansky-Pudlak syndrome (HPS) is a rare genetic disease often complicated by interstitial pneumonia, and there is a lack of effective treatment options available for this condition.
Hermansky-Pudlak syndrome (HPS) is an autosomal recessive hereditary disease that may be complicated by progressive and potentially fatal interstitial pneumonia. We herein report a 64-year-old woman with interstitial pneumonia associated with HPS type 4 whom we treated with nintedanib after pirfenidone proved ineffective. To our knowledge, there have been no previous reports of nintedanib being used to treat a patient with HPS type 4. There is a need for clinical trials of antifibrotic agents, including nintedanib, pirfenidone, and new therapeutic agents with different mechanisms of action in these patients.
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