Internal Medicine
Online ISSN : 1349-7235
Print ISSN : 0918-2918
ISSN-L : 0918-2918
CASE REPORTS
Interstitial Pneumonia Secondary to Hermansky-Pudlak Syndrome Type 4 Treated with Different Antifibrotic Agents
Junko ItanoYasushi TanimotoGoro KimuraNoboru HamadaHisaaki TanakaShinsuke NinomiyaKenjiro KosakiNobuaki MiyaharaYoshinobu MaedaKatsuyuki Kiura
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JOURNALS OPEN ACCESS

2021 Volume 60 Issue 5 Pages 783-788

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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.

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© 2021 by The Japanese Society of Internal Medicine
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