Primary Immunodeficiency and Other Diseases With Immune Dysregulation

Primary immunodeficiency states are not uncommon disorders which can affect the innate or adaptive arms of the immune system. All of these diseases have the potential for adverse pulmonary manifestations, including infections and noninfectious complications. In this chapter, we review the major diseases of the innate and adaptive immune system with a focus on their effects on the lung. Included are descriptions of the disorders, laboratory and diagnostic findings, associated cellular and biochemical effects, genetic abnormalities and inheritance, infectious or noninfectious pulmonary manifestations, and management approaches.