Indicators of cellular and humoral immunity in children with primary ciliary dyskinesia

Abstract

Primary ciliary dyskinesia (PCD) is a rare genetic disorder (belonging to a group of ciliopathies) caused by impaired mucociliary clearance. It leads to chronic bacterial colonization of the respiratory tract and manifests as recurrent respiratory infections. Since clinical manifestations of PCD resemble those of primary immunodeficiencies (PID), this induces diagnostic challenges and can lead to misinterpretation of the causes of frequent respiratory infections. Differential diagnosis of these two disease groups requires a comprehensive laboratory examination including immune status evaluation since patient management and prognosis are ultimately different. In patients with PCD, antibacterial therapy and kinesitherapeutic methods are dedicated to airway clearance from bronchial secretions, whereas substitution therapy is of key importance in PID persons. Aim: to assess cellular and humoral immunity in children with PCD and to determine an effect of potential immune dysregulation on the disease course. Materials and Methods: the study was hosted by Department of Chronic Pediatric Inflammatory and Allergic Diseases at Veltischev Institute. 66 children with a verified diagnosis of primary ciliary dyskinesia who underwent serum immunoglobulin assay and lymphocyte subpopulation pattern evaluation were enrolled. Results: the study did not reveal any significant abnormalities specific for immunodeficiencies. Humoral immunity indicators corresponded to the age norm. Cellular immunity disorders were of a reactive nature due to chronic antigenic stimulation. Conclusion: in PCD patients, recurrent infections are not caused by impaired immune system, but by mucociliary clearance disorders leading to persistent bacterial inflammation. In this regard, management of mucostasis whose appropriate treatment minimizes an effect of the disease on the respiratory system without immunoglobulin replacement therapy, is a key therapeutic approach. This confirms that recurrent respiratory infections in PCD patients are independent of immunodeficiency. KEYWORDS: children, primary ciliary dyskinesia, immunoglobulins, lymphocyte subpopulations, mucociliary clearance, recurrent respiratory infections. FOR CITATION: Mizernitsky Yu.L., Novak A.A., Dyachenko D.P. Indicators of cellular and humoral immunity in children with primary ciliary dyskinesia. Russian Journal of Woman and Child Health. 2026;9(3):348–352 (in Russ.). DOI: 10.32364/2618-8430-2026-9- 3-18