Non-thromboembolic risk in systemic lupus erythematosus associated with antiphospholipid syndrome.

OBJECTIVES: We investigated the impact of secondary antiphospholipid syndrome (APS) and antiphospholipid antibody (aPL) positivity on the non-thromboembolic clinical manifestations of systemic lupus erythematosus (SLE).

METHODS: In total, 224 patients with SLE were studied, of whom 105 were aPL-positive; 52 fulfilled the criteria for APS. SLE- and APS-related clinical and laboratory features were assesed: SLE patients with aPL or APS were compared with those without these features.

RESULTS: Not only thromboembolic events, but also Coombs-positive haemolytic anaemia, thrombocytopenia and endocarditis occurred significantly more frequently in the aPL-positive than in the aPL-negative patients. In the APS + SLE subgroup, several non-thromboembolic symptoms occurred more often than in the absence of APS: pleuritis, interstitial lung disease, myocarditis, nephritis and organic brain syndrome. The mean number of major organ manifestations (1.2 vs. 0.5) and the overall number of organ manifestations (8.1 vs. 6.9) were higher in the APS + SLE patients than in those without APS (p < 0.05). The APS + SLE subgroup more frequently required intensive immunosuppressive treatment than did the APS-negative patients (p < 0.05).

CONCLUSIONS: SLE patients with aPL positivity or secondary APS also have a higher risk to develop non-thromboembolic disease manifestations in addition to the aPL-related symptoms, and are predisposed to more severe SLE manifestations.