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Common Variable Immunodeficiency, Pediatric

Elena E. Perez, MD, PhD Reviewed 10/2018
 


BASICS

DESCRIPTION

  • Common variable immunodeficiency is the most common clinically important primary immunodeficiency syndrome, characterized by

    • Low IgG, IgA, and/or IgM

    • Recurrent infections

    • A wide spectru...

DIAGNOSIS

HISTORY

  • Recurrent sinopulmonary infections, especially sinusitis and pneumonia, with encapsulated bacteria

  • Autoimmune diseases such as autoimmune hemolytic anemia (AIHA), idiopathic thrombocyt...

TREATMENT

MEDICATION

First Line

Immunoglobulin replacement (lifetime) therapy 

Second Line

Antibiotics as needed for infection may also be used as adjunct to immunoglobulin replacement as prophylaxis. 

ADDITIONAL THERAPIES

ONGOING CARE

FOLLOW-UP RECOMMENDATIONS

  • Close and frequent follow-up is warranted for patients with severe, recurrent symptoms. It may be as frequent as monthly, depending on symptoms.

  • Signs and symptoms...

ADDITIONAL READING

  • Azizi G, Abolhassani H, Asgardoon MH, et al. Autoimmunity in common variable immunodeficiency: epidemiology, pathophysiology and management. Expert Rev Clin Immunol.  2017;13(2...

CODES

ICD9

  • 279.06 Common variable immunodeficiency

  • 279.00 Hypogammaglobulinemia, unspecified

  • 279.10 Immunodeficiency with predominant T-cell defect, unspecified

  • 279.06 Common variable immunodeficiency

ICD10

FAQ

  • Q: What is the life expectancy of patients with the diagnosis of common variable immunodeficiency?

  • A: Because the clinical presentations and symptoms are variable, it is difficult to predict the li...

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