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PubMed · 8343021

[C2 deficiency discovered in pneumococcal meningitis].

Abstract

BACKGROUND: Congenital deficiencies of complement system proteins are rare. Patients with C2 deficiency have a high incidence of vascularitis syndromes. Most patients with this deficiency have no problems with increased susceptibility to infection, most commonly due to pneumococci, presumably because of the protective function of the alternative pathway. CASE REPORT: A 22 month-old girl was admitted because of acute meningitis and otitis. She had had 2 episodes of otitis media at the age of 1 year. Analysis of the CSF showed that this meningitis was due to pneumococcal infection. Recovery was complete after 15 days of antibiotic therapy. Total hemolytic complement activity (CH50) was low during the infection; one month later, the CH50 value was about zero as was C2, while C3 and C4 were normal. The patient was given polyvalent pneumococcal and anti-Haemophilus vaccines plus prophylactic penicillin G. Laboratory tests for systemic lupus erythematosus were negative. CONCLUSION: A defect of complement function should be suspected in any patient with severe of recurring pyogenic infections. Complement disorders can be detected one month later by means of the relatively simple hemolytic complement assay.

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BibTeXRIS

M Monfort-Gouraud, M A Le Gall, F Nicot, J Badoual. 1993. [C2 deficiency discovered in pneumococcal meningitis].. https://pubmed.ncbi.nlm.nih.gov/8343021/

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Immunoglobulin deficiencies and susceptibility to infection among homozygotes and heterozygotes for C2 deficiency.

About 25% of C2-deficient homozygotes have increased susceptibility to severe bacterial infections. C2-deficient homozygotes had significantly lower serum levels of IgG2, IgG4, IgD, and Factor B, significantly higher levels of IgA and IgG3 and levels of IgG1 and IgM similar to controls. Type 1 (28 bp deletion in C2 exon 6 on the [HLA-B18, S042, DR2] haplotype or its fragments) and type II (non-type I) C2-deficient patients with increased susceptibility to bacterial infection had significantly lower mean levels of IgG4 (p < 0.04) and IgA (p < 0.01) than those without infections (who had a higher than normal mean IgA level) but similar mean levels of other immunoglobulins and Factor B. Of 13 C2-deficient homozygotes with infections, 85% had IgG4 deficiency, compared with 64% of 25 without infections. IgD deficiency was equally extraordinarily common among infection-prone (50%) and noninfection-prone (70%) homozygous type I C2-deficient patients. IgD deficiency was also common (35%) among 31 type I C2-deficient heterozygotes (with normal or type II haplotypes), but was not found in 5 type II C2-deficient heterozygotes or 1 homozygote. Thus, C2 deficiency itself is associated with many abnormalities in serum immunoglobulin levels, some of which, such as in IgG4 and IgA, may contribute to increased susceptibility to infection. In contrast, IgD deficiency appears not to contribute to increased infections and appears to be a dominant trait determined by a gene or genes on the extended major histocompatibility complex (MHC) haplotype [HLA-B 18, S042, DR2] (but probably not on type II C2-deficient haplotypes) similar to those previously identified on [HLA-B8, SC01, DR3] and [HLA-B18, F1C30, DR3].

Complement C2↗