The physiopathology of the catastrophic antiphospholipid (Asherson's) syndrome: Compelling evidence

Catastrophic antiphospholipid syndrome (CAPS)

“…Several mechanisms have been proposed such as molecular mimicry, infections, activation of endothelium in the microvasculature and microvascular occlusions [3]. Kitchens et al [4] have suggested that the vascular occlusions are themselves responsible for the ongoing thrombosis.…”

“…These molecules can also act on leukocytes and platelets to increase their adhesion to vascular endothelium and to promote microthrombosis and the local release of toxic mediators, including proteases and oxygen-derived free radicals. The interaction between all these cells in the presence of aPL leads to the diffuse microvasculopathy that characterises CAPS and leads to multiorgan failure [3,5,6].…”

Eculizumab Prevents Recurrent Antiphospholipid Antibody Syndrome and Enables Successful Renal Transplantation

“…Whether the binding of APLA is primarily a cause or a consequence of endothelial cell activation is unclear, but complement cascade activation with subsequent C5b-9 deposition leads to the generation of a potently pro-thrombotic environment (16,17). In APS, large vessel thrombosis can be seen, but in CAPS, most of the vascular thrombosis involves the microvasculature, analogous pathologically to prototypic pauci-inflammatory microthrombotic syndromes such as TTP and aHUS.…”

“…In renal transplant recipients, it has been used for the treatment of a variety of complement/ antibody-mediated microangiopathy syndromes including aHUS (24,25) and antibody-mediated rejection (10)(11)(12)(13)(14)(15)(16)(17)(18)(19)(20)(21)(22)(23)(24)(25)(26) and in single case studies of patients with CAPS (13) and APS (27).…”

Antiphospholipid Antibodies and APS Nephropathy

“…1) [3]. This proinflammatory/ prothrombotic state does not induce thrombosis, however, until an inciting factor such as infection or trauma triggers a thrombogenic vascular response [8].…”