Diagnosis and treatment of primary immunodeficiency disease: the role of the otolaryngologist

Abstract

Objective: The objective of the study was to review the diagnosis and treatment of primary immunodeficiency disease (PID) and the role of otolaryngologists in the management of PID.

Methods: A search was conducted of PubMed and the Web sites of organizations for PID patients for literature pertaining to the diagnosis and treatment of PID, with an emphasis on the role of otolaryngologists. The reference lists of selected articles were reviewed for additional articles.

Results: Patients with PID commonly present with respiratory tract infections (eg, recurrent ear, nose, or throat infections) and chest disease. Diagnostic delays or inadequate treatment of PID may lead to significant morbidity and premature mortality. Immunoglobulin (Ig) replacement is the cornerstone of therapy for most patients with PID. Although intravenous Ig is the most popular route of administration in the United States, subcutaneous Ig administration may be appropriate for patients with poor venous access, those who are unable to tolerate intravenous Ig, or those who prefer the independence and flexibility of self-administration.

Conclusions: Recognition and diagnosis of PID by otolaryngologists are critical to optimizing patient outcomes. Several therapeutic regimens for Ig replacement are now available that offer patients increased flexibility and independence.