New diagnostic criteria for common variable immune deficiency (CVID), which may assist with decisions to treat with intravenous or subcutaneous immunoglobulin

Abstract

SummaryCommon variable immune deficiency (CVID) is the most frequent symptomatic primary immune deficiency in adults. The standard of care is 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 paper, 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 hypogammaglobulinaemia are termed hypogammaglobulinaemia of uncertain significance (HGUS). These patients require long-term follow-up, as some will evolve into CVID.

Severe pneumococcal pneumonia following Measles- Mumps- Rubella vaccination / Pneumonie pneumococică severă dezvoltată ca urmare a vaccinării Rujeolă-Oreion-Rubeolă

“…However, the patient reported to have been always in "good health and had never taken antibiotics or was hospitalized for infections before the current event", despite frequent exposure to respiratory pathogens due to her occupational activity as kindergarten teacher. Therefore, one of the criteria of the revised diagnostic definition for "probable CVID" was missing (16). Although increasing evidence suggests that immunoglobulin deficiencies predispose patients to invasive bacterial infections (14), it remains a subject of debate if the patient's immune deficit could have been enough to trigger the pneumococcal infection even in the absence of MMR vaccination history.…”

“…AIDS (16,17). A recently published review summarized the role of impaired immune pathways in influenza virus infection, which induces susceptibility to secondary bacterial pneumonia (20).…”

Is there a need to redefine the diagnostic criteria for common variable immunodeficiency?

“…Editorial Seppänen, Aghamohammadi & Rezaei terms [11][12][13][14]. In the authors' opinion, these patients should not be named CVID.…”

“…Highly likely any immunodeficiency unit having success in increasing the level of public awareness on PIDD in its area will meet many such borderline patients. Some of them may even have a known monogenic defect [11][12][13][14]. During follow-up, some do develop a full-blown CVID phenotype and the delay in treatment becomes minimal.…”

“…In the process, we should not assume that with any PAD diagnosis automatically comes IgGRT. New criteria to define CVID have been recently proposed [12]. To consolidate different criteria and views to interpret vaccine responses, we may need to define a definite CVID patient as someone without known monogenic defects, with severely low vaccine responses and immunoglobulin levels together with classical phenotype.…”