Assessment of complement deficiency in patients with meningococcal diseasein the Netherlands

The frequency of complement deficiency in 176 of 7,732 patients with mening ococcal disease in the Netherlands from 1959 through 1992 was assessed. Com plement deficiency was found in six patients (3%): 3 (7%) of the patients w ith Neisseria meningitidis serogroup C disease, 1 (2%) of the patients with N. meningitidis serogroup A disease, and 2 (33%) of the patients with infe ctions due to uncommon serogroups and nongroupable strains of N, meningitid is. Of 91 additional patients with meningococcal infections due to uncommon serogroups, 33% also had complement deficiency. Thirty-four of the 36 comp lement-deficient patients with meningococcal disease who were from 33 famil ies were 5 years of age or older. Twenty-six additional complement-deficien t relatives were found. Screening individuals with meningococcal disease du e to uncommon serogroups who were 5 years of age or older identified 30 of the 33 complement-deficient families. Only 27% of the complement-deficient relatives had had meningococcal disease. This risk was lower for relatives with properdin deficiency (18%) than for those deficient in the late compon ent of complement (38%). Therefore, pedigree studies are warranted for iden tifying those complement-deficient persons who require vaccination for meni ngococcal disease.