124 - Pulmonary Complications of Primary Immunodeficiencies

Abstract

Primary immune deficiency disorders (PIDDs) are intrinsic defects of the immune system that predispose individuals to infection, malignancy, and autoimmunity. PIDDs are not rare. The lung is commonly involved in PIDDs and is frequently the major organ involved. A diagnostic evaluation for PIDD is a fundamental but often overlooked issue in the evaluation of patients with pulmonary infections. Evaluation of the immune system is warranted in adult patients with two or more radiographically documented cases of pneumonia. However, a single infection of the lung with an opportunistic or unusual pathogen, or a complicated pneumonia, warrants an evaluation for primary and secondary immunodeficiencies. A patient with a first pneumonia, but with a history of intractable sinus disease or recurrent gastrointestinal infection, or with other disorders associated with PIDD (e.g., autoimmunity, intractable eczema, unusual facies) should also be evaluated for a PIDD. The history and clinical presentation of patients with PIDDs suggest the type of immunologic defect present. The onset of disease associated with cellular immunodeficiencies or phagocytic deficiencies usually begins in infancy or early childhood. In contrast, the onset of infections in patients with antibody deficiencies can vary from infancy to late adulthood.