Selective deficiency of immunoglobulin A [IgA]

This code represents a primary immunodeficiency characterized by significantly reduced levels of immunoglobulin A (IgA) in the blood and secretions, while other immunoglobulin levels are typically normal. Patients with this condition may be asymptomatic or experience recurrent infections, autoimmune disorders, or allergic conditions.

Use this code when documentation confirms a diagnosis of selective IgA deficiency, typically based on laboratory findings showing low serum IgA levels. This diagnosis is often made after investigating recurrent sinopulmonary infections, gastrointestinal issues, or autoimmune manifestations.

• Ensure laboratory results explicitly state low IgA levels to support this diagnosis.
• Do not use this code for secondary IgA deficiencies caused by other underlying conditions or medications; instead, code the underlying cause.
• Consider additional codes for any associated clinical manifestations, such as recurrent infections (e.g., J01.90 for acute sinusitis).