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Ten of 12 patients with systemic lupus erythematosus (SLE) who had hemolytic anemia and thrombocytopenic purpura (Evans' syndrome) during their course had evidence of antiphospholipid antibodies either because they had a false positive VDRL test (8 patients), a prolonged partial thromboplastin time (5 patients), a lupus anticoagulant (3/4 patients), and/or anticardiolipin antibodies as determined by an ELISA method (7 patients). Antibodies to cardiolipin were found in very high levels (up to 38 standard deviations above the mean of normal controls) and were of both IgG and IgM isotypes. The 2 patients with SLE and Evans' syndrome who did not have evidence of antiphospholipid antibodies were studied at the onset of SLE which occurred with Evans' syndrome. Although cardiolipin is not a constituent of the cell wall of either platelets or erythrocytes, other phospholipids that cross react antigenically with cardiolipin are and can be exposed through cell damage. This could be a mechanism whereby hemolytic anemia and thrombocytopenia could occur in the same patient with SLE. Whether absorption of the antiphospholipid antibody during the acute episode of hemocytopenia could occur, and thus prevent its detection at such time, remains undetermined.

We studied 500 consecutive patients with systemic lupus erythematosus (SLE) for antibodies to phospholipids (APLA) by an ELISA method using cardiolipin as antigen and antiimmunoglobulins G, M and A to determine their isotype. Once entered into this prospective study the patients were followed for up to 16 months (mean 7.7 +/- 4.72 SD) with periodic determinations of APLA. Of the 500 patients with SLE, 88 had had thrombocytopenia, 25 had had hemolytic anemia, 25 had had both, and 362 had no history of these hemocytopenias. If we considered the odds ratio of these 362 patients for having high titer APLA as 1, patients with a history of thrombocytopenia, hemolytic anemia or both had significantly higher odds ratios of having APLA than did those without hemocytopenia. Patients with thrombocytopenia had significantly higher levels of IgG APLA, those with hemolytic anemia had significantly higher titers of IgM APLA and patients with both had significantly higher titers of both of these APLA isotypes, than did patients without hemocytopenias. A correlation between positive direct Coombs' tests and IgM APLA was also found. We conclude that APLA is associated with these hemocytopenias in SLE. This might be due to their interaction with negatively charged phospholipids in the cell walls of the respective cells.

We studied 199 patients with systemic lupus erythematosus (SLE) who had been at risk for recurrent fetal loss. Thirty-six had had recurrent fetal loss. These patients, plus a group of 38 healthy women with history of recurrent fetal loss and a group of healthy women who had had at least 3 pregnancies, were tested for antiphospholipid antibodies (APLA) of the 3 major immunoglobulin isotypes using an ELISA method with cardiolipin as antigen. Thirty of the 36 patients with SLE with recurrent fetal loss, 23 of the 163 patients with SLE without recurrent fetal loss, 3 of the 38 healthy women with recurrent fetal loss and no healthy woman with normal obstetric histories had APLA. In all but 3 of the patients with SLE with recurrent fetal loss these included APLA of the IgG isotype. Considering the odds ratio of having recurrent fetal loss as 1 for those patients with SLE without APLA, we found it to be 10.54 times higher in patients with SLE with high titer APLA. Titers of IgG APLA were also 3.2 times higher in patients with SLE with recurrent fetal loss than in those without. We conclude that recurrent fetal loss associates strongly with APLA of the IgG isotype.