New diagnostic criteria for Common Variable Immune Deficiency (CVID), which may assist with decisions to treat with intravenous or subcutaneous Immunoglobulin

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1. Rohan Ameratunga^1,2,3,*, 
2. See-Tarn Woon^1,2, 
3. David Gillis⁴, 
4. Wikke Koopmans^1,2,
5. Richard Steele^1,5

DOI: 10.1111/cei.12178

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Clinical & Experimental Immunology

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1. This article has been accepted for publication and undergone full peer review but has not been through the copyediting, typesetting, pagination and proofreading process, which may lead to differences between this version and the Version of Record. Please cite this article as doi:10.1111/cei.12178

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Abstract

Common Variable Immune Deficiency (CVID) is the most frequent symptomatic primary immune deficiency in adults. The standard of care is life-long Intravenous Immunoglobulin (IVIG) or subcutaneous immunoglobulin (scIG) therapy. The cause of CVID is currently unknown and there is no universally accepted definition of CVID. This creates problems in determining which patients will benefit from IVIG/scIG treatment. In this article, we review the difficulties with the commonly used European Society of Immune Deficiencies (ESID) and the Pan American Group for Immune Deficiency (PAGID) definition of CVID. We propose new criteria for the diagnosis of CVID, which are based on recent scientific discoveries. Improved diagnostic precision will assist with treatment decisions including IVIG/scIG replacement. We suggest that asymptomatic patients with mild hypogammaglobulinemia are termed hypogammaglobulinemia of uncertain significance (HGUS). These patients require long-term follow up as some will evolve into CVID.

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