Immunodeficiency Syndromes for the Otolaryngologist

PROGRAM DESCRIPTION: Primary immunodeficiency disorders (PIDDs) are a broad spectrum of diseases with a common feature that the immune system is ineffective, thereby increasing the body's susceptibility to infections. The Immune Deficiency Foundation (IDF) estimates that there are 250,000 people in the US diagnosed with PIDD. Yet, according to the NIH, approximately 500,000 are affected by PIDD, suggesting that there are many who remain undiagnosed. A diagnosis of PIDD is often missed since healthy people also contract infections from time to time. It takes an average of 12.4 years for patients to be diagnosed with PIDD.

One of the most common signs of PIDD is having infections that are more frequent, longer lasting or harder to treat than are the infections of someone with a typical immune system. Signs and symptoms of PIDD can include frequent and recurrent sinus infections and ear infections.

Not all patients respond to standard medical and surgical treatments. Although there are a variety of reasons a patient may be refractory to therapy, one possible etiology is the presence of an underlying immunodeficiency. This session will focus on the description, recognition, and treatment of several antibody deficiencies associated with CRS, including common variable immunodeficiency (CVID), and specific antibody deficiency (SAD). The diagnosis of PIDD in patients with CRS is important because of the large clinical implications it can have on management.

This session will discuss when and how to perform a basic immunodeficiency workup for both pediatric and adult patients presenting with frequent, recurrent, and difficult to treat ear and sinonasal infections emphasizing immunoglobulin quantification and postvaccination antibody titers. We will also discuss how to interpret these immunological testing results and what should be the next steps in management. Given the prevalence of immunodeficiency, ENTs should have a low threshold to consider these diagnoses. Patients sometimes lack timely access to immunologists and ENTs need to understand and provide basic immunodeficiency evaluations to their patients.