Interstitial Pneumonia Secondary to Hermansky-Pudlak Syndrome Type 4 Treated with Different Antifibrotic Agents

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 pirfenid...

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Detalles Bibliográficos
Autores principales: Itano, Junko, Tanimoto, Yasushi, Kimura, Goro, Hamada, Noboru, Tanaka, Hisaaki, Ninomiya, Shinsuke, Kosaki, Kenjiro, Miyahara, Nobuaki, Maeda, Yoshinobu, Kiura, Katsuyuki
Formato: Online Artículo Texto
Lenguaje:English
Publicado: The Japanese Society of Internal Medicine 2020
Materias:
Case Report
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7990633/
https://www.ncbi.nlm.nih.gov/pubmed/33229802
http://dx.doi.org/10.2169/internalmedicine.5493-20
Descripción
Sumario: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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