Immunological abnormalities in primary APS evolving into SLE: 6 years follow-up in women with repeated pregnancy loss

Abstract

We have performed a prospective study to determine the prevalence of immunological abnormalities and the evolution from primary antiphospholipid syndrome (APS) into systemic lupus erythematosus (SLE) in women who had had unexplained repeated pregnancy loss (PL) and APS. Of 105 women with abortions or fetal deaths, 33(31%) fulfilled criteria for APS. Among these patients with primary APS, 24% had antinuclear antibodies (ANA), 91% had elevated circulating immune complexes (CIC), 70% had low total haemolytic complement (CH100), 52% had low levels of complement 4 (C4) and 30% had low levels of complement 3 (C3), in a significantly higher prevalence than women whose pregnancies were successful (control group). Through out a 6 y follow-up, 3 (9%) of the patients with APS who had autoimmune related abnormalities when entered into the study developed features of lupus like disease (LLD) or fullblown SLE. Our findings suggest that women with unexplained repeated PL with APS who presented with positive ANA, high levels of CIC, low levels of CH100, C3 and C4, may define a subset of patients exhibiting immunological alterations similar to those of SLE. These parameters may help in the assessment of prognosis in APS patients with PL. Those patients should be carefully surveyed with regard to the development of connective tissue diseases.

Mechanisms of aPL-mediated thrombosis: Effects of aPL on endothelium and platelets

“…These findings were recently confirmed in rats by Fischetti et al [10•], who showed that thrombus formation induced by antibodies to 2 -GPI requires a priming factor such as bacterial LPS and is complement dependent [10•]. Interestingly, hypocomplementemia has been found in a significant proportion of patients with primary APS, and it was associated with thrombosis in one study and with livedo reticularis and thrombocytopenia in another publication [40][41][42].…”

Avaliação clínico-laboratorial de pacientes com síndrome antifosfolípide primária segundo a frequência de anticorpos antinucleares (FAN Hep-2)

“…Likewise, Carbone et al demonstrated that the presence of ANA in 33 women with PAPS represented a greater risk of evolution of PAPS into SLE in six patients. 26 The association found in the present study between ANA Hep-2 positivity and a higher frequency of aCL antibodies cold suggest a polyclonal activation of lymphocytes in APS. This activation has been demonstrated by the presence of at least 30 different types of autoantibodies related to APS.…”

Defects in the disposal of dying cells lead to autoimmunity

“…Others and we have suggested an important role of complement (mainly C1q) and DNaseI in the clearance process of dying cells and subcellular fragments [18][19][20][21][22][23][24][25][26]. Patients with primary antiphospholipid syndrome who have low levels of the complement components C3 and C4 and low CH 100 values also show immunologic alterations similar to those of SLE [27]. The fast and efficient uptake of dying cells is of main importance to prevent contact of the immune system with intracellular autoantigens.…”