PULMONARY PATHOLOGIES AND MANAGEMENT STRATEGIES IN PRIMARY IMMUNODEFICIENCIES

pulmonary_pathologies_kapak

OBSTRUCTIVE LUNG DISEASESAND PRIMARY IMMUNODEFICIENCY

Ferhat Sağun1 Turgut Teke2

1Konya Necmettin Erbakan University, Faculty of Medicine, Department of Immunology and Allergic Diseases, Konya, Türkiye
2Konya Necmettin Erbakan University, Faculty of Medicine, Department of Chest Diseases, Konya, Türkiye

Sağun F, Teke T. Obstructive Lung Diseases and Primary Immunodeficiency. In: Arslan Ş editor. Pulmonary Pathologies and Management Strategies in Primary Immunodeficiencies. 1st ed. Ankara: Türkiye Klinikleri; 2025. p.61-71.

ABSTRACT

Obstructive lung diseases such as asthma, chronic obstructive pulmonary disease (COPD), bronchiectasis, and bronchiolitis obliterans (BO) are important complications that may develop in individuals with primary immunodeficiency (PID) and can significantly affect quality of life. The development of these conditions is influenced by recurrent respiratory tract infections, chronic inflammation, dysregulation of immune system mechanisms, and genetic predispositions. The frequency and clinical course of these pulmonary diseases may vary among different PID subtypes. In addition to common variable immunodeficiency (CVID), X-linked agammaglobulinemia (XLA), selective IgA deficiency (SIgAD), and combined immunodeficiencies, these conditions have also been described in patients with genetic mutations such as DOCK8, STAT3, NFKB1/NFKB2, CD40L, and IKZF1. Impaired mucosal immunity, continuous exposure of the airways to pathogens, inadequate responses to infections, and post-infectious tissue remodeling processes can lead to irreversible lung damage. Clinical manifestations may range from mild or asymptomatic presentations to prominent symptoms such as chronic cough, dyspnea, and progressive respiratory failure. Diagnostic methods including spirometry, diffusion capacity testing, and high-resolution computed tomography (HRCT) of the chest are commonly used to assess pulmonary involvement. Early recognition of structural lung diseases such as bronchiectasis and BO is essential for preventing permanent damage. Treatment approaches are shaped according to the patient’s immunological and clinical status and include immunoglobulin replacement therapy (IgRT), inhaled corticosteroids, bronchodilators, macrolide prophylaxis, physiotherapy interventions, and appropriate vaccination strategies. Control of infections, management of inflammation, and support of secretion clearance play key roles in preserving airway integrity. When determining vaccination strategies, the patient’s antibody response capacity, genetic background, and immunological profile should be taken into account. Early detection of obstructive lung diseases in PID patients and the implementation of individualized, multidisciplinary follow-up plans constitute a fundamental approach for preserving long-term respiratory health. Collaboration among pulmonology, immunology, and infectious disease disciplines in clinical follow-up is crucial, and a patient-centered approach contributes to treatment success and improved quality of life.

Keywords: Primary immunodeficiency diseases; Lung diseases, obstructive; Bronchiectasis; Pulmonary disease, chronic obstructive; Asthma

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