Immune deficiencies, infection, and systemic immune disordersInfection outcomes in patients with common variable immunodeficiency disorders: Relationship to immunoglobulin therapy over 22 years
Section snippets
Inclusion criteria
Patients that met the CVID criteria30 were selected, namely those with a reduced serum IgG level (<6.0 g/L) and either a serum IgA level <0.8 g/L or a serum IgM level <0.5 g/L or both, over 4 years of age at diagnosis, and exclusion of other conditions or therapies associated with antibody failure. Of these patients with CVIDs, 73 had serum IgG levels <3.5 g/L at diagnosis, and therefore, specific antibody testing was not essential for the diagnosis.29 Of the remaining 17 patients, 12 had
Patient characteristics
One hundred and fifteen patients with confirmed CVIDs and 17 with XLA, seen regularly in the past 25 years (1982-2007), were included initially. Of these, 25 patients with CVIDs were excluded, 10 for noncompliance with therapy, poor infection history or missing serum IgG data, and 15 with less than 1 year available since stabilization of trough IgG. Two patients with XLA were excluded, 1 for noncompliance and 1 for <1 year of follow-up. Patient characteristics are shown in Table I. Diagnostic
Discussion
This is the first study to determine the relationships between doses of replacement therapy, infection rates, and trough IgG levels and to compare these for patients with different clinical CVIDs phenotypes or common complications. Furthermore, this is a substantial group of patients with CVIDs followed over 741 patient-years in 1 center using validated data over 22 years. The policy of adjusting the dose of replacement therapy to reduce the infection rate to a minimum in a given patient has
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Supported by the NIHR Oxford Biomedical Research Centre; unrestricted gifts from Baxter Healthcare, Talecris, and the Jeffrey Modell Foundation; the Centre of Excellence award from the Primary Immunodeficiency Association; and a 7th Framework grant, EURO-PADnet no. 201549, from the European Commission.
Disclosure of potential conflict of interest: S. Misbah is on advisory boards for CSL Behring, Baxter, and Biotest. The rest of the authors have declared that they have no conflict of interest.