TY - JOUR
T1 - Microangiopathic haemolytic anaemia resembling thrombotic thrombocytopenic purpura in systemic lupus erythematosus
T2 - The role of ADAMTS13
AU - Lansigan, Frederick
AU - Isufi, Iris
AU - Tagoe, Clement E.
PY - 2011/5
Y1 - 2011/5
N2 - 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.
AB - 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.
KW - Microangiopathic haemolytic anaemia
KW - Systemic lupus erythematosus
KW - Thrombotic thrombocytopenic purpura
UR - http://www.scopus.com/inward/record.url?scp=79955141428&partnerID=8YFLogxK
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U2 - 10.1093/rheumatology/keq395
DO - 10.1093/rheumatology/keq395
M3 - Review article
C2 - 21149242
AN - SCOPUS:79955141428
VL - 50
SP - 824
EP - 829
JO - Rheumatology and Rehabilitation
JF - Rheumatology and Rehabilitation
SN - 1462-0324
IS - 5
M1 - keq395
ER -