联合肺纤维化和肺气肿所致肺动脉高压中肺血管扩张剂对肺动脉顺应性的改善作用

Improvement of pulmonary arterial compliance by pulmonary vasodilator in pulmonary hypertension from combined pulmonary fibrosis and emphysema.

作者信息

Kusaka Kei, Morio Yoshiteru, Kimura Yuya, Takeda Keita, Kawashima Masahiro, Masuda Kimihiko, Matsui Hirotoshi

机构信息

Center for Pulmonary Circulation and Hemoptysis, Department of Respiratory Medicine, National Hospital Organization Tokyo National Hospital, 3-1-1 Takeoka, Kiyose-shi, Tokyo, 204-8585, Japan.

Center for Pulmonary Diseases, Department of Respiratory Medicine, National Hospital Organization Tokyo National Hospital, 3-1-1 Takeoka, Kiyose-shi, Tokyo, 204-8585, Japan.

出版信息

Respir Med Case Rep. 2019 Sep 27;28:100940. doi: 10.1016/j.rmcr.2019.100940. eCollection 2019.

DOI:10.1016/j.rmcr.2019.100940

PMID:31667076

原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC6812224/

Abstract

Combined pulmonary fibrosis and emphysema (CPFE) is a common but under-recognized syndrome characterized with distinct profiles of both pulmonary fibrosis and emphysema. Pulmonary hypertension (PH) is particularly prone to develop as a common complication, leading to exercise limitation and worse prognosis of CPFE. Although the therapy of patients with PH from CPFE cannot be endorsed, an individual treatment may be considerable when accompanying severe PH. We report a case of a 71-year-old male with PH from CPFE, who improved pulmonary arterial compliance (PAC) and exercise capacity in response to pulmonary vasodilator.

摘要

合并性肺纤维化和肺气肿（CPFE）是一种常见但未被充分认识的综合征，其特征为具有独特的肺纤维化和肺气肿表现。肺动脉高压（PH）作为一种常见并发症特别容易发生，导致运动受限和CPFE预后更差。尽管CPFE合并PH患者的治疗方法尚无定论，但在伴有严重PH时个体化治疗可能是值得考虑的。我们报告一例71岁男性CPFE合并PH患者，其对肺血管扩张剂治疗反应良好，肺动脉顺应性（PAC）和运动能力得到改善。