Microangiopathic haemolytic anaemia resembling thrombotic thrombocytopenic purpura in systemic lupus erythematosus: the role of ADAMTS13

Abstract

Thrombotic thrombocytopenic purpura (TTP) is a rare but frequently fatal complication of SLE. It occurs in the context of both active and inactive lupus and carries a worse overall prognosis than idiopathic acquired TTP. Recent advances in the knowledge and treatment of TTP do not seem to have brought similar improvements in the management and outcome of TTP in SLE. The illumination of the role of the von Willebrand factor multimer protease, ADAMTS13 in idiopathic TTP continues to enhance our comprehension of the pathogenesis of the disease and has contributed to improvements in diagnosis and management. We explore the overlap of TTP and SLE, and discuss the current understanding of the involvement of ADAMTS13 and its implications for patients with this uncommon form of microangiopathic haemolytic anaemia.

Unusual association of lupus and thrombotic thrombocytopenic purpura like syndrome: clinical experience of a rare presentation

“…Although TTP and SLE are different diseases, they may present as overlapping for each other although the association between them hasn't been well defined [4] . There are many hypotheses are being found to explain this association include abnormal endothelial activation, elevated levels of D-dimers, ADAMTS13-resistant von Willebrand Factor and defects in the complement system regulation [5] . SLE can be presented with symptoms resembling those of TTP making the differentiation very difficult, especially our patient didn't have any history or symptoms suggesting lupus before [3] .…”

Anemia hemolítica microangiopática en lupus eritematoso sistémico: revisión narrativa de la literatura

“…Pero algunas poblaciones son más susceptibles de desarrollarla, incluyendo los pacientes con LES (1 a 4%) 9 .…”

Thrombotic thrombocytopenic purpura as an initial presentation of systemic lupus erythematosus with acquired ADAMTS 13 antibody

“…The mainstay of treatment in TTP is plasma exchange 1 7. Even with treatment, SLE-associated TTP was reported to have a higher mortality, 34–62.5%, compared to idiopathic TTP, which was about 20% mortality 1 4–6 8 9. Some small studies showed a benefit of adding other immunosuppressive agents in SLE-associated TTP 9.…”