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

Frederick Lansigan, Iris Isufi, Clement E. Tagoe

Research output: Contribution to journalReview article

35 Scopus citations

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 languageEnglish (US)
Article numberkeq395
Pages (from-to)824-829
Number of pages6
JournalRheumatology
Volume50
Issue number5
DOIs
StatePublished - May 1 2011

Keywords

  • Microangiopathic haemolytic anaemia
  • Systemic lupus erythematosus
  • Thrombotic thrombocytopenic purpura

ASJC Scopus subject areas

  • Rheumatology
  • Pharmacology (medical)

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