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支气管扩张症与系统性硬化症之间新出现的关联:评估患病率和潜在因果关系。

The emerging association between bronchiectasis and systemic sclerosis: assessing prevalence and potential causality.

机构信息

Department of General Medicine, Fiona Stanley Hospital, Perth, Western Australia, Australia.

Institute for Health Research, University of Notre Dame, Fremantle, Western Australia, Australia.

出版信息

Intern Med J. 2023 Jul;53(7):1204-1211. doi: 10.1111/imj.15661. Epub 2022 Nov 5.

DOI:10.1111/imj.15661
PMID:34894170
Abstract

BACKGROUND

Bronchiectasis has been observed in association with systemic sclerosis (SSc). Theorised aetiology includes aspiration related to oesophageal dysmotility, immunosuppressant medication use and the direct effect of collagen deposition on airway calibre.

AIMS

To detail bronchiectasis prevalence in an SSc population who have had a high-resolution computed tomography (HRCT) of the thorax. We assessed whether oesophageal dysmotility, demographic variables, SSc duration or subclass were associated with bronchiectasis.

METHODS

Participants in the Australian Scleroderma Cohort Study (ASCS) with a HRCT were included. The ASCS provided demographic and clinical data. HRCT studies were reviewed for bronchiectasis, oesophageal dilatation and interstitial lung disease (ILD). Traction bronchiectasis associated with ILD was recorded as a separate entity to bronchiectasis. Oesophageal dysmotility was defined by symptoms and/or oesophageal dilatation.

RESULTS

Of the 256 participants, 16.4% (n = 42) had bronchiectasis. Logistic regression analysis revealed no significant association between bronchiectasis and oesophageal dysmotility (observed in 95.7%), any demographic variable, SSc duration or subclass. A negative association between bronchiectasis and ILD was observed (P = 0.009; odds ratio 0.322; 95% confidence intervals 0.137-0.756).

CONCLUSION

Those with SSc appear to have an increased risk for bronchiectasis. Since bronchiectasis was not more frequent in participants with a longer duration of SSc, we hypothesise that its development is not related to immunosuppression alone. Oesophageal dysmotility was almost universal in our population such that its effect on bronchiectasis development could not be concluded. A negative association between bronchiectasis and ILD reflects that bronchiectasis occurring alongside ILD was recorded as a separate entity.

摘要

背景

支气管扩张症与系统性硬化症(SSc)有关。其理论发病机制包括与食管动力障碍相关的吸入、免疫抑制剂的使用以及胶原蛋白沉积对气道直径的直接影响。

目的

详细描述曾进行高分辨率胸部计算机断层扫描(HRCT)的 SSc 人群中支气管扩张症的患病率。我们评估了食管动力障碍、人口统计学变量、SSc 持续时间或亚型是否与支气管扩张症有关。

方法

纳入澳大利亚硬皮病队列研究(ASCS)中进行 HRCT 的参与者。ASCS 提供了人口统计学和临床数据。对 HRCT 研究进行了支气管扩张症、食管扩张和间质性肺病(ILD)的评估。与 ILD 相关的牵引性支气管扩张症被记录为支气管扩张症的一个单独实体。食管动力障碍通过症状和/或食管扩张来定义。

结果

在 256 名参与者中,16.4%(n=42)患有支气管扩张症。逻辑回归分析显示,支气管扩张症与食管动力障碍(95.7%观察到)、任何人口统计学变量、SSc 持续时间或亚型之间无显著关联。观察到支气管扩张症与 ILD 呈负相关(P=0.009;比值比 0.322;95%置信区间 0.137-0.756)。

结论

SSc 患者似乎有更高的支气管扩张症风险。由于 SSc 持续时间较长的患者中支气管扩张症的发生率并没有更高,我们假设其发展不仅仅与免疫抑制有关。在我们的人群中,食管动力障碍几乎普遍存在,因此无法得出其对支气管扩张症发展的影响。支气管扩张症与 ILD 之间的负相关反映了与 ILD 同时发生的支气管扩张症被记录为一个单独的实体。

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