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以气道为中心的间质性纤维化伴平滑肌增生及重度肺动脉高压。

Airway-centered Interstitial Fibrosis Involving Smooth Muscle Hyperplasia with Severe Pulmonary Hypertension.

作者信息

Gocho Kyoko, Kimura Tokuhiro, Hamanaka Nobuyuki, Ishii Toshiharu, Takemura Tamiko, Shimizu Kunihiko

机构信息

Department of Respiratory Medicine, Saiseikai Yokohamashi Tobu Hospital, Japan.

Department of Diagnostic Pathology, Saiseikai Yokohamashi Tobu Hospital, Japan.

出版信息

Intern Med. 2020 Aug 15;59(16):2029-2034. doi: 10.2169/internalmedicine.4199-19. Epub 2020 Jun 2.

DOI:10.2169/internalmedicine.4199-19
PMID:32493848
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC7492125/
Abstract

In the 2013 updated classification of the American Thoracic Society/European Respiratory Society, airway-centered interstitial fibrosis (ACIF) is included as a bronchiolocentric pattern of interstitial pneumonia (IP) among idiopathic IPs. We encountered a case of severe pulmonary hypertension (PH) with chronic IP. The patient initially presented with shortness of breath and often lost consciousness due to PH, and seven years after his first visit, he ultimately died. An autopsy revealed ACIF and usual IP. In particular, the ACIF comprised non-atypical smooth muscle hyperplasia, and pulmonary hypertensive vascular degeneration was detected. This case may represent a new pathological feature of ACIF.

摘要

在2013年美国胸科学会/欧洲呼吸学会更新的分类中，气道中心性间质纤维化（ACIF）被纳入特发性间质性肺炎（IP）中的细支气管中心型间质性肺炎模式。我们遇到一例伴有慢性IP的重度肺动脉高压（PH）病例。该患者最初表现为呼吸急促，常因PH而失去意识，首次就诊7年后最终死亡。尸检显示有ACIF和普通型IP。特别是，ACIF包括非典型平滑肌增生，并检测到肺动脉高压性血管退变。该病例可能代表了ACIF的一种新的病理特征。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/251c/7492125/9404dfb68f1c/1349-7235-59-2029-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/251c/7492125/a53437d689ec/1349-7235-59-2029-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/251c/7492125/1ed823f56b78/1349-7235-59-2029-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/251c/7492125/4c934a748aba/1349-7235-59-2029-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/251c/7492125/9404dfb68f1c/1349-7235-59-2029-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/251c/7492125/a53437d689ec/1349-7235-59-2029-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/251c/7492125/1ed823f56b78/1349-7235-59-2029-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/251c/7492125/4c934a748aba/1349-7235-59-2029-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/251c/7492125/9404dfb68f1c/1349-7235-59-2029-g004.jpg

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Pulmonary vascular and cardiac impairment in interstitial lung disease.间质性肺疾病中的肺血管和心脏损害
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