A comparison of thrombotic thrombocytopenic purpura in an inception cohort of patients with and without systemic lupus erythematosus

Abstract

Despite early and more aggressive therapy in sTTP, mortality was higher and the time to complete remission were longer, suggesting that sTTP is more severe. The tempo of development of TTP in SLE patients was slower.

Rituximab was effective on refractory thrombotic thrombocytopenic purpura but induced a flare of hemophagocytic syndrome in a patient with systemic lupus erythematosus

“…Moreover, inhibitors of ADAMTS-13 activity have been found in immunoglobulin (Ig) G from patients with TTP. Impaired ADMTS-13 activity and the presence of inhibitory autoantibodies to ADAMTS-13 have been also reported in patients with TTP in SLE, some of whom were treated successfully with rituximab [12,13,15]. In contrast to these reports, ADAMTS-13 activity was normal, and B-cell depletion therapy was effective on TTP in our case.…”

“…Recently, the effectiveness of rituximab has been reported [11]. As in our case, the effectiveness of rituximab on TTP associated with SLE has been reported [12][13][14][15]. Therefore, B-cell depletion therapy could be effective for treating refractory TTP in SLE.…”

Thrombotic microangiopathy due to multiple autoantibodies related to antiphospholipid syndrome

“…Patients with SLE are likely to develop multiple autoantibodies causing TMA, such as anti-ADAMTS13 antibody, anti-antiphospholipid antibody, anticardiolipin antibody, and anti-factor H antibody, which may accelerate endothelial damage and thrombotic events [12,13]. Additionally, despite early and intensive therapy, mortality is higher in adults who have TTP with SLE compared with patients with idiopathic TTP [14]. Acute renal involvement of APS includes renal artery or vein thrombosis, renal infarction, APS nephropathy (membranous nephropathy), and TMA.…”

Treg cells in thrombotic thrombocytopenic purpura associated with systemic lupus erythematosus patients

“…1 Although plasma exchange dramatically improved the prognosis of TTP with over 80% of survival rate, 2 the episode will be severe and lethal (from 34.1 to 62.5% mortality rate) when TTP occurs in patients with SLE(referred as sTTP). 3,4 In the last two decades, autoimmunity or vasculopathy with endothelial damage and platelet aggregation was considered as the pathogenesis of TTP in the setting of SLE. 4,5 In recent years, an autoimmunity mechanism was proposed to be more important to the onset of TTP in the context of SLE, which was supported by the presence of various antibodies such as anti-endothelial cell antibody, antiplatelets antibody, 3 and anti-ADAMTS13 (von Willebrand factor cleaving metalloprotease) antibody.…”

“…3,4 In the last two decades, autoimmunity or vasculopathy with endothelial damage and platelet aggregation was considered as the pathogenesis of TTP in the setting of SLE. 4,5 In recent years, an autoimmunity mechanism was proposed to be more important to the onset of TTP in the context of SLE, which was supported by the presence of various antibodies such as anti-endothelial cell antibody, antiplatelets antibody, 3 and anti-ADAMTS13 (von Willebrand factor cleaving metalloprotease) antibody. 6 However, the presence of various antibodies is still unclear until now.…”