Although functional hyposplenism, secondary to Fc-receptor blockage by
circulating immune complexes saturation, has been described in system
ic lupus erythematosus (SLE), only few cases of complete asplenism hav
e been reported. We observed a 60-year-old woman with congenital asple
nism who presented with active SLE. The course and the clinical charac
teristics of such patients are reviewed and the relationship between t
he asplenic state and initiation and severity of SLE are discussed. Th
ese patients are at high risk for fatal pneumococcemia and pneumococca
l vaccine is recommended even if long term results are still conflicti
ng.