This practice parameter for the diagnosis and management of primary immunodeficiency (PIDD) is intended to provide a practical guide for the clinical recognition and diagnosis of immunodeficiency, along with general principles that guide management of these disorders. Among the recommendations are:

• PIDD has a prevalence of 1:2000 children.

• PIDD is subdivided into humoral or antibody deficiencies, and combined immunodeficiency.

• Initial evaluation is guided by the clinical presentation, and screening tests are applied and followed by advanced tests, ensuring efficient and thorough evaluation of mechanisms of immune dysfunction that underlie the clinical presentation.

• Diagnosis and therapy should be guided overall or performed in consultation with persons and centers with knowledge and experience diagnosing and treating a broad range of immunodeficiencies.

Citation: Bonilla FA, Khan DA, Ballas ZK, et al. Practice parameter for the diagnosis and management of primary immunodeficiency. J Allergy Clin Immunol. [Published online ahead of print September 11, 2015]. doi: 10.1016/j.jaci.2015.04.049.

Commentary: Clinicians should be aware of PIDD in order to refer appropriate patients on to an allergist/immunologist for further evaluation. While many different types of PIDD exist, they generally present with recurrent or severe infections or infections by unusual organisms. For example, approximately a quarter of patients older than 2 years of age with invasive pneumococcal disease have an identifiable PIDD. This is a group of disorders that, while rare, are helpful to be aware of and to refer on for further evaluation when indicated. —Neil Skolnik, MD