reticular basement membrane
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Reticular basement membrane (RBM) thickening may occur in children with allergic bronchial asthma (BA), cystic fibrosis (CF), and primary ciliary dyskinesia (PCD). Its functional consequences remain unknown. We investigated the relationship between baseline RBM thickness and subsequent spirometry. In our cohort follow-up study, patients aged 3-18 yr with BA, CF, and PCD and controls underwent baseline lung clearance index (LCI) measurement, spirometry, and endobronchial biopsy sampling. Total RBM and collagen IV-positive layer thickness were measured. Trends in forced vital capacity (FVC), forced expired volume in 1 s (FEV1), and FEV1/FVC were analyzed during follow-up, and their relationship to baseline characteristics was studied using univariate analysis and multiple regression models. Complete baseline data were available in 19 patients with BA, 30 patients with CF, 25 patients with PCD, and 19 controls. The RBM was thicker in patients with BA (6.33 ± 1.22 μm), CF (5.60 ± 1.39 μm), and PCD (6.50 ± 1.87 μm) than in controls (3.29 ± 0.55 μm) (all P < 0.001). The LCI was higher in patients with CF (15.32 ± 4.58, P < 0.001) and PCD (10.97 ± 2.46, P = 0.002) than in controls (7.44 ± 0.43). The median follow-up times were 3.6, 4.8, 5.7, and 1.9 years in patients with BA, CF, PCD, and controls, respectively. The z-scores of FEV1 and FEV1/FVC deteriorated significantly in all groups except in controls. In patients with CF and PCD, trends in FEV1z-scores correlated with baseline LCI and RBM; in BA, it correlated with collagen IV. In multiple regression models, RBM morphology and ventilation inhomogeneity could predict up to 84.4% of variability in spirometry trends. In conclusion, baseline LCI value and RBM morphology may predict trends in subsequent spirometry.NEW & NOTEWORTHY This paper deals with the relationship between reticular basement membrane (RBM) morphology at baseline and follow-up spirometry in children with asthma, cystic fibrosis, and primary ciliary dyskinesia. For the first time, to our knowledge, the possibility to predict subsequent lung function development using selected baseline characteristics (reticular basement membrane morphology from endobronchial biopsy and ventilation inhomogeneity from nitrogen multiple breath washout test) is proposed. Corresponding predictive models are presented.
- MeSH
- bazální membrána patologie MeSH
- bronchiální astma * patologie MeSH
- cystická fibróza * patologie MeSH
- dítě MeSH
- kolagen MeSH
- lidé MeSH
- následné studie MeSH
- plíce patologie MeSH
- poruchy ciliární motility * MeSH
- spirometrie MeSH
- usilovný výdechový objem MeSH
- zánět patologie MeSH
- Check Tag
- dítě MeSH
- lidé MeSH
- Publikační typ
- časopisecké články MeSH
- práce podpořená grantem MeSH
BACKGROUND: Bronchial epithelial reticular basement membrane (RBM) thickening occurs in diseases with both eosinophilic (allergic bronchial asthma [BA]) and neutrophilic (cystic fibrosis [CF] and primary ciliary dyskinesia [PCD]) chronic airway inflammation; however, the lung function and airway remodeling relation remains unclear. The aim of this study was to test whether ventilation inhomogeneity is related to RBM thickening. METHODS: Multiple breath washout test, endobronchial biopsy, and BAL were performed in 24 children with CF, 11 with PCD, 15 with BA, and in 19 control subjects. Lung clearance index at 2.5% (1/40th) of starting nitrogen concentration (LCI2.5), RBM thickness, and lavage fluid cytology were quantified; their mutual associations were studied by using Spearman rank correlations (r). RESULTS: In asthma, ventilation inhomogeneity (mean ± SD) was mild (LCI2.5, 9.3 ± 1.4 vs 7.9 ± 0.9 in control subjects; P = .0391), and the RBM thickened (5.26 ± 0.98 μm vs 3.12 ± 0.62 μm in control subjects; P < .0001). No relation between RBM thickness and ventilation inhomogeneity or lavage cytology was found. In CF and PCD, RBM thickness was similar to that in asthma (4.54 ± 0.66 μm and 5.27 ± 1.11 μm, respectively), but ventilation inhomogeneity was significantly higher (LCI2.5, 12.5 ± 2.4 and 11.8 ± 2.5). Both in CF and PCD, RBM thickness correlated with LCI2.5 (r = 0.594, P = .015; r = 0.821, P = .023). In PCD only, RBM thickness was also related to the number of neutrophils in lavage fluid (r = 0.821; P = .023). CONCLUSIONS: Lung function impairment in relation to RBM thickness was milder in BA than in CF and PCD. In asthma, ventilation inhomogeneity did not correlate with RBM thickness, whereas it did in CF and PCD. This outcome suggests a different structure-function relation in these diseases.
- MeSH
- bazální membrána patologie MeSH
- biopsie metody MeSH
- bronchiální astma * patologie patofyziologie MeSH
- bronchoalveolární lavážní tekutina MeSH
- bronchoskopie MeSH
- bronchy * patologie patofyziologie MeSH
- cystická fibróza * patologie patofyziologie MeSH
- dítě MeSH
- korelace dat MeSH
- lidé MeSH
- mukociliární clearance MeSH
- neutrofily patologie MeSH
- plicní ventilace fyziologie MeSH
- poruchy ciliární motility * patologie patofyziologie MeSH
- remodelace dýchacích cest MeSH
- respirační funkční testy metody MeSH
- Check Tag
- dítě MeSH
- lidé MeSH
- mužské pohlaví MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- práce podpořená grantem MeSH
Účel přehledu Z pohledu klasické medicíny se astma a chronická obstrukční plicní nemoc (CHOPN) vyznačují odlišnými klinickými, fyziologickými a patologickými rysy. Nezřídka však nemocní vykazují vzájemně se překrývající klinické příznaky a patofyziologické abnormality – u pacientů trpících těžkým astmatem se můžeme setkat s trvalou obstrukcí dýchacích cest a u pacientů trpících CHOPN můžeme pozorovat hyperreaktivitu a eozinofilii. Podobné zánětlivé a strukturální rysy se vyskytují i na úrovni patologických nálezů a mohou souviset s fenotypovým překrýváním. Nové poznatky U pacientů trpících astmatem dochází na úrovni zánětu k překrývání příznaků společných s CHOPN, jako je zvýšená četnost neutrofilů u nemocných s těžkým astmatem nebo vztah mezi T‑buňkami CD8+ a zhoršováním plicních funkcí. U CHOPN může minimalizace eozinofilie významně přispívat k omezení výskytu exacerbací. Obě nemoci jsou provázeny strukturálními změnami, které ovšem postihují různé oddíly dýchacích cest odlišně. Při obou onemocněních dochází ke změnám v epitelu dýchacích cest, k depozici extracelulární matrix a k hypertrofii hlenových žlázek. Astmatici mají tlustší retikulární bazální membránu a vykazují výraznější abnormality hladké svaloviny, zatímco svébytným rysem CHOPN je emfyzém. Souhrn Rozpoznání odlišných a podobných rysů obou onemocnění na úrovni patologických nálezů by mohlo vést k lepšímu poznání překrývajících se klinických a patofyziologických fenotypů a tím i k lepšímu plánování specifické léčby a dlouhodobé péče o nemocné.
Classically, asthma and chronic obstructive pulmonary disease present distinct clinical, physiologic and pathologic features. However, not infrequently, patients may present with overlapping clinical symptoms and physiological abnormalities: patients with severe asthma may present with fixed airway obstruction and patients with chronic obstructive pulmonary disease may have hyperresponsiveness and eosinophilia. At pathological level, inflammatory and structural similarities also occur and may be related to the phenotypic overlaps. RECENT FINDINGS: In patients with asthma overlaps at inflammatory level exist with chronic obstructive pulmonary disease, such as increased neutrophilia in patients with severe asthma or an association of CD8+ T cells and lung-function decline. In chronic obstructive pulmonary disease, minimizing eosinophilia may be important to reduce exacerbations. Structural alterations occur in both diseases, but involving airway compartments differently. Airway epithelial changes, extracellular matrix deposition and mucus gland hypertrophy occur in both diseases. Asthmatics have thicker reticular basement membrane and more prominent smooth-muscle abnormalities, whereas emphysema is a distinct feature of chronic obstructive pulmonary disease. SUMMARY: Recognizing the differences and similarities at pathological level in both diseases may lead to a better understanding of the overlapping clinical and physiological phenotypes, thereby helping to better plan specific treatment and long-term management
