Function and Structure Relationships With Inflammation Differ in Two Chronic Suppurative Lung Diseases.

IF 2.3 3区医学 Q1 PEDIATRICS

Pediatric Pulmonology Pub Date : 2026-05-01 DOI:10.1002/ppul.71659

Teresa Fuchs, Jacqueline Donovan, Andrew Ives, Samantha Irving, Gwyneth Davies, Claire Hogg, Eric Alton, Gemma Wilson, Andrew Bush, Jane C Davies

{"title":"Function and Structure Relationships With Inflammation Differ in Two Chronic Suppurative Lung Diseases.","authors":"Teresa Fuchs, Jacqueline Donovan, Andrew Ives, Samantha Irving, Gwyneth Davies, Claire Hogg, Eric Alton, Gemma Wilson, Andrew Bush, Jane C Davies","doi":"10.1002/ppul.71659","DOIUrl":null,"url":null,"abstract":"Rationale: Cystic fibrosis (CF) and primary ciliary dyskinesia (PCD) are characterized by neutrophilic airway inflammation but differ in clinical features.Objectives: We investigated relationships of pulmonary and systemic inflammatory markers with functional and structural lung disease.Methods: Systemic (CRP, IgG, IL-6, and IL-8) and sputum (calprotectin, IL-6, and IL-8) markers were measured at baseline. Relationships were explored with spirometry, lung clearance index and lung computed tomography (CT) scores at baseline and with spirometry 10 years later.Results: 42 patients (21 CF, 21 PCD) of similar age (median CF 27 y [12-59], PCD 27 y [11-62]) and lung function (median ppFEV1CF 59% [40-99], PCD 67% [30-101]) were enrolled in 2009. Systemic inflammation was significantly higher in CF (Neutrophils, p < 0.05, IL-6 and 8, p < 0.01). Conversely, sputum IL-6 and 8 were higher in PCD (p < 0.01). In CF, sputum IL-6 counter-intuitively correlated with better CT scores (r = -0.51/p < 0.05) at baseline and with better spirometry (r = 0.58, p < 0.05) 10 years later. Conversely, high sputum IL-6 at baseline was associated with worse lung function in PCD (r = 0.81/p = 0.001) after 10 years.Conclusion: This study emphasizes the differences of functional and structural aspects in both diseases. Markers that predict long-term outcome in PCD and in CF were identified. The most striking and unanticipated finding was that sputum IL-6 correlated with better CT scores and lung function in CF. This paradox merits further research but challenges whether sputum IL-6 in CF is always a bad prognostic indicator.","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":"61 5","pages":"e71659"},"PeriodicalIF":2.3000,"publicationDate":"2026-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Pediatric Pulmonology","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.1002/ppul.71659","RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q1","JCRName":"PEDIATRICS","Score":null,"Total":0}

引用次数: 0

Abstract

Rationale: Cystic fibrosis (CF) and primary ciliary dyskinesia (PCD) are characterized by neutrophilic airway inflammation but differ in clinical features.

Objectives: We investigated relationships of pulmonary and systemic inflammatory markers with functional and structural lung disease.

Methods: Systemic (CRP, IgG, IL-6, and IL-8) and sputum (calprotectin, IL-6, and IL-8) markers were measured at baseline. Relationships were explored with spirometry, lung clearance index and lung computed tomography (CT) scores at baseline and with spirometry 10 years later.

Results: 42 patients (21 CF, 21 PCD) of similar age (median CF 27 y [12-59], PCD 27 y [11-62]) and lung function (median ppFEV₁CF 59% [40-99], PCD 67% [30-101]) were enrolled in 2009. Systemic inflammation was significantly higher in CF (Neutrophils, p < 0.05, IL-6 and 8, p < 0.01). Conversely, sputum IL-6 and 8 were higher in PCD (p < 0.01). In CF, sputum IL-6 counter-intuitively correlated with better CT scores (r = -0.51/p < 0.05) at baseline and with better spirometry (r = 0.58, p < 0.05) 10 years later. Conversely, high sputum IL-6 at baseline was associated with worse lung function in PCD (r = 0.81/p = 0.001) after 10 years.

Conclusion: This study emphasizes the differences of functional and structural aspects in both diseases. Markers that predict long-term outcome in PCD and in CF were identified. The most striking and unanticipated finding was that sputum IL-6 correlated with better CT scores and lung function in CF. This paradox merits further research but challenges whether sputum IL-6 in CF is always a bad prognostic indicator.

查看原文本刊更多论文

两种慢性化脓性肺疾病与炎症的功能和结构关系不同。

理由：囊性纤维化（CF）和原发性纤毛运动障碍（PCD）以中性粒细胞性气道炎症为特征，但临床特征不同。目的：探讨肺部和全身炎症标志物与功能性和结构性肺部疾病的关系。方法：在基线时测定全身（CRP、IgG、IL-6、IL-8）和痰液（钙保护蛋白、IL-6、IL-8）标志物。探讨了基线时肺活量测定、肺清除率指数和肺计算机断层扫描（CT）评分与10年后肺活量测定的关系。结果：2009年入组42例患者（21例CF， 21例PCD），年龄相近（中位CF 27岁[12-59]，PCD 27岁[11-62]），肺功能相近（中位ppFEV1CF 59% [40-99]， PCD 67%[30-101]）。结论：本研究强调了两种疾病在功能和结构方面的差异。确定了预测PCD和CF长期预后的标志物。最令人震惊和意想不到的发现是，CF患者的痰IL-6与更好的CT评分和肺功能相关。这一悖论值得进一步研究，但对CF患者的痰IL-6是否总是一个不良预后指标提出了挑战。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

求助全文

约1分钟内获得全文求助全文

来源期刊

Pediatric Pulmonology 医学-呼吸系统

CiteScore

6.00

自引率

12.90%

发文量

468

审稿时长

3-8 weeks

期刊介绍： Pediatric Pulmonology (PPUL) is the foremost global journal studying the respiratory system in disease and in health as it develops from intrauterine life though adolescence to adulthood. Combining explicit and informative analysis of clinical as well as basic scientific research, PPUL provides a look at the many facets of respiratory system disorders in infants and children, ranging from pathological anatomy, developmental issues, and pathophysiology to infectious disease, asthma, cystic fibrosis, and airborne toxins. Focused attention is given to the reporting of diagnostic and therapeutic methods for neonates, preschool children, and adolescents, the enduring effects of childhood respiratory diseases, and newly described infectious diseases. PPUL concentrates on subject matters of crucial interest to specialists preparing for the Pediatric Subspecialty Examinations in the United States and other countries. With its attentive coverage and extensive clinical data, this journal is a principle source for pediatricians in practice and in training and a must have for all pediatric pulmonologists.