Anti-IgA antibodies in common variable immunodeficiency (CVID): diagnostic workup and therapeutic strategy.
Common Variable Immunodeficiency (CVID) patients who are seropositive for anti-IgA antibodies have a predisposition for anaphylactoid reactions to intravenous immunoglobulin replacement therapy (IVIG). Among 88 CVID patients, we identified eight with IgG anti-IgA antibodies (9%). All eight completely lacked IgA (<0.0009 g/l). Five of them had a history of anaphylactoid reactions to IVIG. However, four of these five patients tolerated subcutaneous immunoglobulin replacement therapy (SCIG). To identify predisposing factors for anti-IgA antibodies and related anaphylactoid reactions, we analyzed the clinical and immunological phenotype of affected patients. All eight IgG anti-IgA-positive patients lacked IgA(+) B cells in peripheral blood. Moreover, CVID patients with retained class-switched CD27(pos) IgM(neg) IgD(neg) memory B cells (Freiburg classification group II) and total IgA deficiency seem to have an increased risk for developing anti-IgA antibodies. In seven of the eight patients, lymphoproliferation was observed (most prominently nodular lymphatic hyperplasia), two had granulomatous disease, and two showed autoimmune phenomena.
Horn
Julia
J
Division of Rheumatology and Clinical Immunology, University Hospital Freiburg, Hugstetterstr. 55, D-79106 Freiburg, Germany.
Thon
Vojtech
V
Bartonkova
Dana
D
Salzer
Ulrich
U
Warnatz
Klaus
K
Schlesier
Michael
M
Peter
Hans-Hartmut
HH
Grimbacher
Bodo
B
D000328:Adult; D000707:Anaphylaxis / Q000276:immunology; D000888:Antibodies, Anti-Idiotypic / Q000097:blood*; D017074:Common Variable Immunodeficiency / Q000175:diagnosis* / Q000276:immunology / Q000628:therapy*; D005260:Female; D006801:Humans; D007070:Immunoglobulin A / Q000276:immunology*; D007074:Immunoglobulin G / Q000097:blood; D016756:Immunoglobulins, Intravenous; D008297:Male; D008875:Middle Aged
D000888:Antibodies, Anti-Idiotypic; D007070:Immunoglobulin A; D007074:Immunoglobulin G; D016756:Immunoglobulins, Intravenous