放射学胸膜肺纤维弹性组织增生伴非结核分枝杆菌肺病的独特临床特征：一项多中心回顾性队列研究。

Distinctive clinical features of radiological pleuroparenchymal fibroelastosis with nontuberculous mycobacterial pulmonary disease: A multicenter retrospective cohort study.

机构信息

Department of Medicine, Division of Pulmonary Medicine, Keio University School of Medicine, Tokyo, Japan.

Department of Medicine, Division of Pulmonary Medicine, Keio University School of Medicine, Tokyo, Japan; Department of Clinical Medicine (Laboratory of Bioregulatory Medicine), Kitasato University School of Pharmacy, Tokyo, Japan; Department of Respiratory Medicine, Kitasato University, Kitasato Institute Hospital, Tokyo, Japan.

出版信息

Int J Infect Dis. 2024 Nov;148:107233. doi: 10.1016/j.ijid.2024.107233. Epub 2024 Sep 4.

DOI:10.1016/j.ijid.2024.107233

PMID:39241955

Abstract

OBJECTIVES

To compare the characteristics and prognosis of patients with nontuberculous mycobacterial (NTM) pulmonary disease (PD) with pleuroparenchymal fibroelastosis (PPFE) with those of patients with nodular/bronchiectatic (NB) and fibrocavitary (FC) NTM-PD.

METHODS

This multicenter, retrospective, observational study enrolled 32 patients with NTM-PPFE (median age: 70.5 years, 15 females) from six institutions in Japan from January 2003 to December 2018. Their clinical characteristics and response to therapy were compared with age- and sex-matched cohorts of patients with noncavitary NB and cavitary NB/FC NTM-PD.

RESULTS

Patients with NTM-PPFE had a lower body mass index and a higher standard NTM-PD therapy initiation rate than patients with other NTM-PD types. Sputum culture conversion rates were comparable between groups; however, patients with NTM-PPFE had a higher incidence of treatment-related adverse events, including optic neuropathy associated with high-dose ethambutol therapy, lower percent predicted forced vital capacity values, higher serum Krebs von den Lungen-6 (KL-6) levels, and poorer treatment outcomes than the other groups. Cox regression revealed that NTM-PPFE was an independent risk factor for death/pneumothorax (adjusted hazard ratio: 35.3, 95% confidence interval: 3.90-4692).

CONCLUSION

NTM-PPFE is a unique NTM-PD phenotype with a poorer prognosis than the NB and FC phenotypes.

摘要

目的

比较伴有胸膜肺弹力纤维增生症（PPFE）的非结核分枝杆菌（NTM）肺病（PD）患者与结节/支气管扩张型（NB）和纤维空洞型（FC）NTM-PD 患者的特征和预后。

方法

本多中心、回顾性、观察性研究纳入了 2003 年 1 月至 2018 年 12 月期间日本 6 家机构的 32 例 NTM-PPFE 患者（中位年龄：70.5 岁，女性 15 例）。将他们的临床特征和治疗反应与年龄和性别匹配的非空洞性 NB 和空洞性 NB/FC NTM-PD 患者进行比较。

结果

与其他 NTM-PD 类型的患者相比，NTM-PPFE 患者的体重指数较低，标准 NTM-PD 治疗起始率较高。各组间痰培养转换率相当；然而，NTM-PPFE 患者治疗相关不良反应的发生率较高，包括与大剂量乙胺丁醇治疗相关的视神经病变、用力肺活量预计值百分比较低、血清 Krebs von den Lungen-6（KL-6）水平较高以及治疗结局较差，与其他组相比。Cox 回归分析显示，NTM-PPFE 是死亡/气胸的独立危险因素（调整后的危险比：35.3，95%置信区间：3.90-4692）。