Systemic lupus erythematosus-associated catastrophic antiphospholipid syndrome occurring after typhoid fever - A possible role of Salmonella lipopolysaccharide in the occurrence of diffuse vasculopathy-coagulopathy

We report a case of well-documented typhoid fever in a 30-year-old woman wi th inactive systemic lupus erythematosus with asymptomatic lupus anticoagul ant and high-titer anticardiolipin antibody (aCL), Despite prompt eradicati on of the Salmonella typhi obtained,vith appropriate antibiotic therapy, mu ltiple organ system dysfunction occurred, The central nervous system was in volved, with ischemic infarcts in the occipital lobes. High-dose corticoste roid therapy failed to improve the neurologic manifestations, which respond ed to repeated plasmapheresis. A sharp fall in aCL and anti-beta(2)-glycopr otein I antibody titers was recorded before the start of plasmapheresis, At the same time, IgM and IgG antibodies to Salmonella group O:9 lipopolysacc haride became detectable; the IgM antibodies disappeared within 4 months, w hereas the IgG antibodies remained detectable during the next 13 months, De spite treatment with high-dose corticosteroids and cyclophosphamide, rapidl y progressive glomerulonephritis developed, leading to chronic renal failur e, There is convincing evidence of a link between the S typhi infection and the ensuing catastrophic syndrome in this patient, probably precipitated b y bacterial antigens.