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.
| Original language | English (US) |
|---|---|
| Article number | keq395 |
| Pages (from-to) | 824-829 |
| Number of pages | 6 |
| Journal | Rheumatology |
| Volume | 50 |
| Issue number | 5 |
| DOIs | |
| State | Published - May 2011 |
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Keywords
- Microangiopathic haemolytic anaemia
- Systemic lupus erythematosus
- Thrombotic thrombocytopenic purpura
ASJC Scopus subject areas
- Rheumatology
- Pharmacology (medical)
Cite this
Microangiopathic haemolytic anaemia resembling thrombotic thrombocytopenic purpura in systemic lupus erythematosus : The role of ADAMTS13. / Lansigan, Frederick; Isufi, Iris; Tagoe, Clement E.
In: Rheumatology, Vol. 50, No. 5, keq395, 05.2011, p. 824-829.Research output: Contribution to journal › Article
}
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
UR - http://www.scopus.com/inward/citedby.url?scp=79955141428&partnerID=8YFLogxK
U2 - 10.1093/rheumatology/keq395
DO - 10.1093/rheumatology/keq395
M3 - Article
C2 - 21149242
AN - SCOPUS:79955141428
VL - 50
SP - 824
EP - 829
JO - Rheumatology
JF - Rheumatology
SN - 1462-0324
IS - 5
M1 - keq395
ER -