How primary immune deficiencies impact the lung in childrens

Abstract

Primary immune de�iciencies (PID) encompasse a heterogeneous group of genetically determined disorders that affect development and/or function of innate or adaptive immunity. Consequently, patients with PID suffer from recurrent and/or severe infections that frequently involve the lung.Pulmonary complications, which are largely dependent on the components of the immune system that are defective, cause signi�icant morbidity and mortality. Even though the pattern of lung damage and the types of microorganisms involved may provide insights into potential defects in innate, humoral, or cell-mediated immunity, there is a substantial overlap in lung complications among the different types of PID. Consequently, thorough laboratory investigations are necessary to establish a de�initive diagnosis and to prompt appropriate treatment. Furthermore, the identi�ication of a large number of PID-causing genes allows early, even presymptomatic diagnosis, thus representing an essential tool for prevention of lung damage. This article describes the most common forms of PID, their cellular and molecular bases, and the associated lung abnormalities, and reports on available treatment.