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

doi:10.2169/internalmedicine.5493-20

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

Center for Medical Genetics

Research output: Contribution to journal › Article › peer-review

2 Citations (Scopus)

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 language	English
Pages (from-to)	783-788
Number of pages	6
Journal	Internal Medicine
Volume	60
Issue number	5
DOIs	https://doi.org/10.2169/internalmedicine.5493-20
Publication status	Published - 2021 Mar 1

Keywords

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

ASJC Scopus subject areas

Internal Medicine

Access to Document

10.2169/internalmedicine.5493-20

Cite this

@article{be9878f1923c4bd38bc5e16090cf75b7,

title = "Interstitial pneumonia secondary to Hermansky-Pudlak syndrome type 4 treated with different antifibrotic agents",

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.",

keywords = "Antifibrotic agents, Hermansky-Pudlak syndrome type 4, Idiopathic pulmonary fibrosis, Interstitial pneumonia, Nintedanib, Pirfenidone",

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

note = "Publisher Copyright: {\textcopyright} 2021 The Japanese Society of Internal Medicine",

year = "2021",

month = mar,

day = "1",

doi = "10.2169/internalmedicine.5493-20",

language = "English",

volume = "60",

pages = "783--788",

journal = "Internal Medicine",

issn = "0918-2918",

publisher = "Japanese Society of Internal Medicine",

number = "5",

}

TY - JOUR

T1 - Interstitial pneumonia secondary to Hermansky-Pudlak syndrome type 4 treated with different antifibrotic agents

AU - Itano, Junko

AU - Tanimoto, Yasushi

AU - Kimura, Goro

AU - Hamada, Noboru

AU - Tanaka, Hisaaki

AU - Ninomiya, Shinsuke

AU - Kosaki, Kenjiro

AU - Miyahara, Nobuaki

AU - Maeda, Yoshinobu

AU - Kiura, Katsuyuki

PY - 2021/3/1

Y1 - 2021/3/1

N2 - 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.

AB - 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.

KW - Antifibrotic agents

KW - Hermansky-Pudlak syndrome type 4

KW - Idiopathic pulmonary fibrosis

KW - Interstitial pneumonia

KW - Nintedanib

KW - Pirfenidone

UR - http://www.scopus.com/inward/record.url?scp=85102187978&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=85102187978&partnerID=8YFLogxK

U2 - 10.2169/internalmedicine.5493-20

DO - 10.2169/internalmedicine.5493-20

M3 - Article

C2 - 33229802

AN - SCOPUS:85102187978

SN - 0918-2918

VL - 60

SP - 783

EP - 788

JO - Internal Medicine

JF - Internal Medicine

IS - 5

ER -

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

Abstract

Keywords

ASJC Scopus subject areas

Access to Document

Other files and links

Fingerprint

Cite this