Antibodies and clinical features of the antiphospholipid syndrome as criteria for systemic lupus erythematosus

Abstract

The antiphospholipid syndrome (APS) can occur as a primary diagnosis or as a prominent feature of other diseases, predominantly systemic lupus erythematosus (SLE). The 1982 revised criteria for SLE were published prior to many of the studies which have illuminated current understanding of the antiphospholipid syndrome and several current clinical criteria for SLE, when arising from thrombotic damage to different organ systems, could be attributed to APS, leading to some confusion about where the diagnoses of these two disorders should begin and end. Additionally, APS is a significant generalized risk factor for irreversible organ damage and overall mortality in SLE patients and genetic linkages to HLA in APS hold up whether the disorder is primary or linked to SLE. It is increasingly recognized that APS itself is a complex, heterogenous disorder, involving a spectrum of autoantibodies to phospholipid-binding proteins, many of which have known coagulation-regulating functions. Although the combination of more than one antiphospholipid-related antibody might indicate a more severe phenotype, it is not suggested here that additive criteria for the diagnosis of SLE be accumulated with more than one of these pathologically related autoantibodies. Patients with multiple criteria for APS should be considered to have severe APS but it would be recommended to restrict APS-attributed criteria for SLE to a maximal of two: one immunologic and one clinical. Thus people meeting the Sapporo criteria for APS could gain only a maximum of two criteria for SLE, regardless of how many autoantibodies were detected or how severe the clinical syndrome might be. This would allow manifestations of fullblown APS an appropriate impact towards the diagnosis of SLE without leading to a premature diagnosis of SLE for people who might better be considered to have moderate to severe primary APS.