İnterstisyel Akciğer Hastalığı Tanısı ile İzlenen Hastaların İmmünolojik Özelliklerinin Değerlendirilmesi

Abstract

Childhood Interstitial Lung Diseases (chILD) are a heterogeneous group of chronic respiratory diseases with usually no identifiable etiology. This disease group is rare and exhibits heterogeneous characteristics, showing diffuse pulmonary involvement. In this study, we aimed to investigate certain immunological components that could play a role in the pathogenesis of patients diagnosed with chILD and followed at Hacettepe University Pediatric Pulmonology Unit. Within this scope, regulatory T cell analysis was conducted in the peripheral blood of patients, and the expression levels of IL-10 and TGFB were evaluated intracellularly in these cells. Additionally, natural lymphoid cell characterization (ILC1, ILC2, ILC3) and helper T cell-17 (Th-17) analysis were performed. Intracellular vimentin and periplakin expression were evaluated. A total of 20 patients and 19 controls were included. There was no significant difference in terms of age and gender between patient and control groups. The mean age of the patients was determined to be 14.25 ± 4.7 years, and a consanguinity rate of 40% was observed among patients. Malnutrition was detected in 30% of the patients. The median age of symptom onset was determined to be 48 months, with dyspnea, tachypnea, and cough being the most common presenting symptoms. There was no significant difference between patient and healthy control groups in terms of Treg cell percentage (7.58 ± 5 and 6.2 ± 3.61, respectively), percentage of TGF-β expressing cells evaluated intracellularly in Treg cells (31.08 ± 16.02 and 33.16 ± 14.47, respectively), and IL-10 expressing cell percentage (0.98 ± 2.63 and 0.41 ± 0.56, respectively) (P>0.05). There was no significant difference in Th17 percentage between patient and healthy control groups (21.21 ± 11.05 and 19.29 ± 8.68, respectively) (P=0.29). There was no significant difference between patient and healthy control groups in terms of intracellular periplakin and vimentin expression (p>0.05). After excluding bronchiolitis obliterans patients, a new group was formed (other ILD; n=14), and a significant difference in terms of ILC2 was found between this group and the control group (2.58 ± 2.24 and 4.5 ± 2.59, respectively; P=0.037), but no difference was found in other parameters. There was no significant correlation between study parameters and BMI, FEV1%, FVC%, 6-minute walk test, age at diagnosis, symptom duration, clinical findings, and physical examination findings (P>0.05). Our study is a pilot study evaluating the immunological characteristics in chILD. In order to fully understand the significance of our results, especially a larger patient group and bronchoalveolar lavage samples are needed to support the findings.