Interstitial pneumonia secondary to Hermansky-Pudlak syndrome type 4 treated with different antifibrotic agents

Junko Itano, Yasushi Tanimoto, Goro Kimura, Noboru Hamada, Hisaaki Tanaka, Shinsuke Ninomiya, Kenjiro Kosaki, Nobuaki Miyahara, Yoshinobu Maeda, Katsuyuki Kiura

Research output: Contribution to journalArticlepeer-review

Abstract

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.

Original languageEnglish
Pages (from-to)783-788
Number of pages6
JournalInternal Medicine
Volume60
Issue number5
DOIs
Publication statusPublished - Mar 1 2021

Keywords

  • Antifibrotic agents
  • Hermansky-Pudlak syndrome type 4
  • Idiopathic pulmonary fibrosis
  • Interstitial pneumonia
  • Nintedanib
  • Pirfenidone

ASJC Scopus subject areas

  • Internal Medicine

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