Treatment of Pulmonary Manifestations of Primary Immunodeficiency Diseases

Abstract

Pulmonary complications are the most common presentations in primary immunodeficiency diseases (PIDs). Treatment of pulmonary manifestations of PIDs varies depending on the underlying processes (e.g., humoral, cellular, combined immunodeficiencies) and etiologies (e.g., infectious, inflammatory, malignant, etc.). The management consists of: (a) Prophylactic treatments such as chronic antimicrobial therapy; appropriate vaccinations; immunoglobulin supplementation, mainly in humoral or combined humoral and cellular immunodeficiencies; or anti-inflammatory therapy in diseases with airway involvement (b) Therapeutic management such as appropriate antibiotic coverage for pulmonary infectious manifestations, bronchodilator therapy for obstructive lung disease, airway clearance devices in bronchiectasis, lung transplantation in few cases, and finally bone marrow transplantation as a more definitive therapy in some disorders (c) Strategies to improve symptoms and overall quality of life, for example, cough suppressants, supplemental oxygen therapy, pulmonary rehabilitation, and nutritional support. Here, we provide an overview of different therapeutic and prophylactic approaches based on the underlying immunologic process.