Acute exacerbation of combined pulmonary fibrosis and emphysema associated with Hermansky–Pudlak syndrome

A 30‐year‐old male smoker with congenital amblyopia and oculocutaneous albinism was admitted to our hospital complaining of progressive dyspnea on exertion. Chest computed tomography images revealed diffuse reticular opacities and honeycombing in the bilateral lower lobes with sparing of the subpleu...

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Main Authors:	Sugino, Keishi, Gocho, Kyoko, Kikuchi, Naoshi, Shibuya, Kazutoshi, Uekusa, Toshimasa, Homma, Sakae
Format:	Online
Language:	English
Published:	John Wiley and Sons Inc. 2015
Online Access:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4722101/

id	pubmed-4722101
recordtype	oai_dc
spelling	pubmed-47221012016-02-02 Acute exacerbation of combined pulmonary fibrosis and emphysema associated with Hermansky–Pudlak syndrome Sugino, Keishi Gocho, Kyoko Kikuchi, Naoshi Shibuya, Kazutoshi Uekusa, Toshimasa Homma, Sakae Case Reports A 30‐year‐old male smoker with congenital amblyopia and oculocutaneous albinism was admitted to our hospital complaining of progressive dyspnea on exertion. Chest computed tomography images revealed diffuse reticular opacities and honeycombing in the bilateral lower lobes with sparing of the subpleural region along with emphysema predominantly in the upper lobes. Lung biopsy specimens showed a mixture of usual interstitial pneumonia and a non‐specific interstitial pneumonia pattern with emphysema. Of note, cuboidal epithelial cells with foamy cytoplasm on the alveolar walls and phagocytic macrophages with ceroid pigments in the fibrotic lesions were observed. The patient was diagnosed with Hermansky–Pudlak syndrome (HPS) associated with combined pulmonary fibrosis and emphysema (CPFE). Six years following the patient's initial admission to our hospital, he died from acute exacerbation (AE) of CPFE associated with HPS. This is one of only few reports available on the clinicopathological characteristics of AE in CPFE associated with HPS. John Wiley and Sons Inc. 2015-12-09 /pmc/articles/PMC4722101/ /pubmed/26839694 http://dx.doi.org/10.1002/rcr2.141 Text en © 2015 The Authors. Respirology Case Reports published by John Wiley & Sons Ltd on behalf of The Asian Pacific Society of Respirology. This is an open access article under the terms of the Creative Commons Attribution‐NonCommercial (http://creativecommons.org/licenses/by-nc/4.0/) License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited and is not used for commercial purposes.
repository_type	Open Access Journal
institution_category	Foreign Institution
institution	US National Center for Biotechnology Information
building	NCBI PubMed
collection	Online Access
language	English
format	Online
author	Sugino, Keishi Gocho, Kyoko Kikuchi, Naoshi Shibuya, Kazutoshi Uekusa, Toshimasa Homma, Sakae
spellingShingle	Sugino, Keishi Gocho, Kyoko Kikuchi, Naoshi Shibuya, Kazutoshi Uekusa, Toshimasa Homma, Sakae Acute exacerbation of combined pulmonary fibrosis and emphysema associated with Hermansky–Pudlak syndrome
author_facet	Sugino, Keishi Gocho, Kyoko Kikuchi, Naoshi Shibuya, Kazutoshi Uekusa, Toshimasa Homma, Sakae
author_sort	Sugino, Keishi
title	Acute exacerbation of combined pulmonary fibrosis and emphysema associated with Hermansky–Pudlak syndrome
title_short	Acute exacerbation of combined pulmonary fibrosis and emphysema associated with Hermansky–Pudlak syndrome
title_full	Acute exacerbation of combined pulmonary fibrosis and emphysema associated with Hermansky–Pudlak syndrome
title_fullStr	Acute exacerbation of combined pulmonary fibrosis and emphysema associated with Hermansky–Pudlak syndrome
title_full_unstemmed	Acute exacerbation of combined pulmonary fibrosis and emphysema associated with Hermansky–Pudlak syndrome
title_sort	acute exacerbation of combined pulmonary fibrosis and emphysema associated with hermansky–pudlak syndrome
description	A 30‐year‐old male smoker with congenital amblyopia and oculocutaneous albinism was admitted to our hospital complaining of progressive dyspnea on exertion. Chest computed tomography images revealed diffuse reticular opacities and honeycombing in the bilateral lower lobes with sparing of the subpleural region along with emphysema predominantly in the upper lobes. Lung biopsy specimens showed a mixture of usual interstitial pneumonia and a non‐specific interstitial pneumonia pattern with emphysema. Of note, cuboidal epithelial cells with foamy cytoplasm on the alveolar walls and phagocytic macrophages with ceroid pigments in the fibrotic lesions were observed. The patient was diagnosed with Hermansky–Pudlak syndrome (HPS) associated with combined pulmonary fibrosis and emphysema (CPFE). Six years following the patient's initial admission to our hospital, he died from acute exacerbation (AE) of CPFE associated with HPS. This is one of only few reports available on the clinicopathological characteristics of AE in CPFE associated with HPS.
publisher	John Wiley and Sons Inc.
publishDate	2015
url	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4722101/
_version_	1613527016964882432

Acute exacerbation of combined pulmonary fibrosis and emphysema associated with Hermansky–Pudlak syndrome

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