结缔组织未分化发育不良综合征患者的哮喘：病程特点还是相互加重的机制？

Asthma in patients with the syndrome of undifferentiated dysplasia of connective tissue: peculiarities of the course or mutually aggravating mechanisms?

机构信息

KYIV MEDICAL UNIVERSITY, KYIV, UKRAINE.

NATIONAL PIROGOV MEMORIAL MEDICAL UNIVERSITY, VINNYTSIA, UKRAINE.

出版信息

Wiad Lek. 2024;77(4):821-827. doi: 10.36740/WLek202404130.

DOI:10.36740/WLek202404130

PMID:38865642

Abstract

OBJECTIVE

Aim: To analyse laboratory and biochemical features of the severe persistent course of asthma in patients with undifferentiated connective tissue dysplasia (UCTD) syndrome, and their phenotypic and visceral stigmas of dysembryogenesis.

PATIENTS AND METHODS

Materials and Methods: We enrolled 60 male patients with asthma, aged from 23 to 62 years (mean age (46.83 ±0.85) years): 30 patients with the background of UCTD, and 30 - without UCTD. We analysed clinical, somatometric, surveying (original questionnaire based on the phenotypic map of Glesby), instrumental (spirography, echocardiography, endoscopy, esophagofibrogastroduodenoscopy) and laboratory (including eosinophilic granulocytes and aldosterone levels) data.

RESULTS

Results: Correlations were found in men with UCTD between the number of UCTD markers and rate of earlobe diagonal fold (r=+0.75; р<0.05), asthenic constitution (r=+0.72; р<0.05), easy bruising (r=+0.7; p<0.05) and straight abdominal line hernia (r=+0.52; p<0.05). Average aldosterone serum level in patients with UCTD (176,10 ±11,22) was significantly higher than in those without UCTD (142,77 ±9,43), (p<0.05), as well as average eosinophils levels (1.3 ±0.25 vs. 0.57 ±0.12, p<0.05). In the absolute majority of patients with UCTD (93.3%) asthma onset was confirmed after pneumonia, and their age of asthma manifestation was significantly higher (37.2 ±1.21) than in patients without UCTD (21.4 ±1.13). Also, in patients with UCTD there was a high number of severe exacerbations during the last year (2.7 ±0.12 per year) on the background of high doses of combined inhaled glucocorticosteroids use.

CONCLUSION

Conclusions: Identified "phenotypic profile", clinical and biochemical features of patients with asthma on the background of UCTD syndrome, which determine the severe course and early formation of asthma complications, will further accelerate the diagnosis of this asthma phenotype and improve approaches to the selection of treatment regimens for these patients.

摘要

目的

分析未分化结缔组织病（UCTD）综合征患者中哮喘严重持续病程的实验室和生化特征，以及其胚胎发育障碍的表型和内脏特征。

患者和方法

我们纳入了 60 名男性哮喘患者，年龄 23 至 62 岁（平均年龄（46.83±0.85）岁）：30 名患者存在 UCTD 背景，30 名患者不存在 UCTD 背景。我们分析了临床、人体测量学、调查（基于 Glesby 表型图谱的原始问卷）、仪器（肺活量测定法、超声心动图、内窥镜检查、食管胃十二指肠镜检查）和实验室（包括嗜酸性粒细胞和醛固酮水平）数据。

结果

在存在 UCTD 的男性中，UCTD 标志物数量与耳垂对角线褶皱（r=+0.75；р<0.05）、虚弱体质（r=+0.72；р<0.05）、容易瘀伤（r=+0.7；р<0.05）和直腹疝（r=+0.52；р<0.05）之间存在相关性。存在 UCTD 的患者平均血清醛固酮水平（176.10±11.22）明显高于不存在 UCTD 的患者（142.77±9.43），（р<0.05），以及平均嗜酸性粒细胞水平（1.3±0.25 与 0.57±0.12，р<0.05）。在绝大多数存在 UCTD 的患者（93.3%）中，哮喘发作是在肺炎之后确诊的，并且他们的哮喘发病年龄明显更高（37.2±1.21），而不存在 UCTD 的患者（21.4±1.13）。此外，在存在 UCTD 的患者中，在高剂量联合吸入糖皮质激素使用的背景下，去年严重恶化的次数很高（每年 2.7±0.12 次）。