Nagaroor Anton, Höller Sylvia, Franzen Daniel P
Department of Pulmonology, University Hospital Zurich, Zurich, 8091, Switzerland.
Department of Internal Medicine, Uster Hospital, Brunnenstrasse 46, Uster, 8610, Switzerland.
Sci Rep. 2025 Mar 25;15(1):10210. doi: 10.1038/s41598-024-83240-7.
Idiopathic subglottic stenosis (iSGS) is a rare disease that primarily affects young and middle-aged females. There is a lack of data investigating the pathological mechanisms of this disease. This study aimed to provide histological and immunohistochemical evidence of the differences between specimens from iSGS patients and healthy individuals. Using histological staining, tracheal mucosa specimens from patients with iSGS and healthy individuals were assessed based on abnormal changes, such as fibrosis, acute inflammation, chronic inflammation, perivascular inflammation, and glandular inclusions. Immunohistochemical analyses were performed using Giemsa, CD20 antibody B cell, CD5, CD4, CD8, CD56, CD68, CD25, TIA-1 and Granzyme B staining. For the control group, tracheal biopsies were obtained during bronchoscopic procedures conducted for diagnostic or therapeutic purposes unrelated to iSGS. These individuals did not have tracheal stenosis and were considered healthy in terms of their tracheal condition. Informed consent for the collection of samples for research purposes was obtained from all control group participants prior to the procedure. Thirteen patients with iSGS and five healthy participants were included. A comparison of immune cell populations between the iSGS and control groups revealed significant differences. Lymphocytes were numerously present in 77% of the cases in the iSGS group and few in 60% of the cases in the control group (p = 0.006). In 12 out of 13 (92.31%) cases, we observed inflammatory infiltrates, mainly CD4 T cells and a few cytotoxic CD8 T cells. B cells were present in 11 out of 13 (84.62%) cases but were mostly found in smaller numbers than T cells. Follicle formation was observed in only 2 out of 13 (15.38%) samples. We were not able to detect obvious immunohistochemical features in our patients with iSGS. However, some standard features were present within the samples showing a clear inflammatory state. Patients with iSGS showed a notably higher proportion of lymphocytes compared to control participants. The increase in lymphocytes was largely driven by T cells, with CD4-positive T-helper cells being more numerous than cytotoxic T cells. The clinical history of the patient collective was heterogeneous and did not allow for any conclusions to be drawn regarding the aetiology. Further studies are needed to characterize the tissues to clearly define the aetiology of iSGS.
特发性声门下狭窄(iSGS)是一种罕见疾病,主要影响中青年女性。目前缺乏对该疾病病理机制的研究数据。本研究旨在提供iSGS患者与健康个体标本差异的组织学和免疫组化证据。通过组织学染色,根据纤维化、急性炎症、慢性炎症、血管周围炎症和腺性包涵体等异常变化,对iSGS患者和健康个体的气管黏膜标本进行评估。采用吉姆萨染色、CD20抗体B细胞、CD5、CD4、CD8、CD56、CD68、CD25、TIA - 1和颗粒酶B染色进行免疫组化分析。对于对照组,在进行与iSGS无关的诊断或治疗目的的支气管镜检查过程中获取气管活检标本。这些个体没有气管狭窄,就气管状况而言被认为是健康的。在操作前,已从所有对照组参与者处获得用于研究目的的样本采集知情同意书。纳入了13例iSGS患者和5名健康参与者。iSGS组与对照组免疫细胞群体的比较显示出显著差异。淋巴细胞在iSGS组77%的病例中大量存在,而在对照组60%的病例中数量较少(p = 0.006)。在13例中的12例(92.31%)病例中,观察到炎症浸润,主要是CD4 T细胞和少量细胞毒性CD8 T细胞。B细胞在13例中的11例(84.62%)病例中存在,但大多数量少于T细胞。仅在13例中的2例(15.38%)样本中观察到滤泡形成。在iSGS患者中我们未能检测到明显的免疫组化特征。然而,在显示明显炎症状态的样本中存在一些标准特征。与对照组参与者相比,iSGS患者的淋巴细胞比例显著更高。淋巴细胞的增加主要由T细胞驱动,CD4阳性辅助性T细胞比细胞毒性T细胞数量更多。患者群体的临床病史各异,无法就病因得出任何结论。需要进一步研究对组织进行特征化,以明确界定iSGS的病因。