Anticardiolipin antibodies (aCL) and HLA-DR antigens were determined in 314 central European patients with systemic lupus erythematosus (SLE). Both HLA-DR4 and DR7 were increased in aCL-positive patients, and aCL were significantly associated with DRw53. The association between DRw53 and aCL was also apparent in those 17 patients with SLE and the anticardiolipin syndrome. There was no association between aCL and HLA-DQ or C4 alleles in SLE.

In a multicentre study anticardiolipin antibodies of the IgG and IgM isotypes were measured by a solid phase enzyme immunoassay in 368 patients with systemic lupus erythematosus (SLE) who were not selected on the basis of features of antiphospholipid syndrome. Clinical and laboratory associations of increased levels of anticardiolipin antibodies were evaluated. IgG and IgM antibodies to cardiolipin were documented in 224 (60.9%) and 128 (34.8%) patients, respectively. Regarding the symptoms of antiphospholipid syndrome, elevated amounts of anticardiolipin IgG were significantly associated with spontaneous abortion (P < 0.001), thrombocytopenia (P < 0.01), livedo reticularis (P < 0.01) and a positive direct Coombs test (P < 0.05), but not with thrombosis or central nervous system diseases such as epilepsy and psychosis. IgM antibodies to cardiolipin were associated with a positive direct Coombs test (P < 0.01), but with no other symptom of antiphospholipid syndrome. The predictive values of anticardiolipin antibody determinations in unselected SLE patients were poor for all features of antiphospholipid syndrome because of high proportions of false-positive and false-negative results. As for other manifestations of SLE, positive correlations between raised antibodies to double-stranded DNA and the occurrence of anticardiolipin antibodies of the IgG isotype were observed, and anticardiolipin IgM was negatively associated with nephritis.

Patients with antiphospholipid syndrome (APS) are prone to thromboembolism. So far, the only predictive parameters for further complications are their number in patient's history and perhaps the titre of aPL. Derived from clinical investigation of stroke and obvious analogies between cerebrovascular ischemia (CVI) in patients with carotid artery disease (CAD) and patients with APS, a novel non-invasive method is introduced using transcranial Doppler (TCD) long-term monitoring to detect high energy ultrasonic signals (so called ‘microemboli’) in the cerebral vasculature. In patients with CAD, these microemboli proved to correlate with past and impending symptoms of CVI permitting therapeutic stratification by their detectability. In SLE and APS, this technique enabled identification of very similar signals in cerebral bloodstream of APS patients. Microemboli were highly associated with the history of CVI and the titre of aPL. Detection of microemboli offers new possibilities in risk estimation, therapeutic stratification and in studying pathophysiology of APS.

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