Journal
CLINICAL IMMUNOLOGY
Volume 145, Issue 3, Pages 241-250Publisher
ACADEMIC PRESS INC ELSEVIER SCIENCE
DOI: 10.1016/j.clim.2012.09.007
Keywords
C3 deficiency; Neisserial infection; C4 nephritic factor; Autoimmunity
Categories
Funding
- NIH [RO1 AI041592, T32 HL007317]
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C3 deficiency is a rare disorder that leads to recurrent pyogenic infections. Here we describe a previously healthy 18 y/o Caucasian male with severe meningococcal disease. Total hemolytic activity was zero secondary to an undetectable C3. The C3 gene was normal by sequencing. Mixing the patients serum with normal human serum led to C3 consumption. An IgG autoantibody in the patients serum was identified that stabilized the classical pathway C3 and C5 convertases, thus preventing decay of these enzyme complexes. This autoantibody is an example of a C4 nephritic factor, with an additional feature of stabilizing the C5 convertase. Previous patients with C4 nephritic factor had membranoproliferative glomerulonephritis. Two years after presentation, this patient's C3 remains undetectable with no evidence of renal disease. We revisit the role of autoantibodies to classical pathway convertases in disease, review the literature on C4-NeF and comment on its detection in the clinical laboratory. (C) 2012 Elsevier Inc. All rights reserved.
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