Thrombosis in Systemic Lupus Erythematosus

Abstract

Systemic lupus erythematosus (SLE) is a potentially fatal multiorgan inflammatory disease that primarily affects females. Due to the heterogeneity of clinical manifestations and lack of laboratory tests that are both specific and sensitive for the disease, diagnosis of SLE can often be difficult. Although the precise etiology remains to be fully elucidated, it is probable that various environmental, genetic, and hormonal factors contribute to the development of the disease. Patients with SLE have an increased risk for premature thrombosis and/or atherosclerosis, with up to half experiencing a thrombotic event. Furthermore, antiphospholipid antibodies probably play a key role in the development of thrombosis by affecting various hemostatic protein interactions with phospholipids and cell surfaces as well as platelet function. Despite recent advances in knowledge related to the factors that contribute to the pathophysiology of SLE, numerous challenges related to earlier diagnosis as well as the prediction and prevention of thrombotic events remain to be fully addressed.

IgG glycan hydrolysis by endoglycosidase S diminishes the proinflammatory properties of immune complexes from patients with systemic lupus erythematosus: A possible new treatment?

“…It is well established that ICs are involved in many of the disease manifestations of SLE, including nephritis (3,5), pleuritis (6), vasculitis (7), skin and CNS involvement (8)(9)(10), and thrombosis (11,12). Deposited IgG or ICs cause local inflammation by activation of the classical pathway of complement with recruitment of PMNs, which could contribute to tissue destruction.…”

“…Many of the disease manifestations of SLE, including nephritis (3,5), pleuritis (6), vasculitis (7), and skin and central nervous system (CNS) involvement (8)(9)(10) are most likely IC mediated. Furthermore, autoantibodies against phospholipids are associated with the development of thrombosis (11,12). If not deposited in tissue, ICs may be phagocytosed by plasmacytoid dendritic cells (PDCs) and induce the production of IFN␣ (13,14), which is a key cytokine in the development and progression of SLE (15) and breaking of self tolerance leading to autoimmunity (16)(17)(18).…”

Oral Direct Anticoagulants in Thrombosis Management in Anti-Phospholipid Syndrome: Unanswered Questions

“…Possible mechanisms include the effects of inflammatory activity and aPLs [3]. aPLs might increase the risk of VTE through various mechanisms, including affecting reactants involved in coagulation [4].…”

The antiphospholipid syndrome: a large elephant with many parts or an elusive chameleon disguised by many colours?

“…However, a proportion of APS patients have another associated disease entity (including SLE; [7]), and may therefore be identified as having "secondary" APS (SAPS), though this terminology may be changing as discussed in the next section. In addition, some PAPS patients may develop features of definite SLE over a period of time [8], and it is therefore recommended that PAPS patients be clinically and serologically monitored for such potential development [4].…”

“…Undoubtedly, this issue will be extensively discussed at the forthcoming 2010 APLA meeting in Texas. 7 Autoimmun Highlights (2010) 1:5-14 A sensitive but relatively nonspecific test for APS. Can be performed in a β2GPI-dependent or β2GPI-independent manner, with the former being more specific for APS.…”