title: 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.
authors.affiliation: Department of Medicine, Division of Rheumatology, Montefiore Medical Center, 111 East 210th Street, Bronx, NY 10467-2490, USA.
authors.forename: Frederick
authors.initials: F
authors.lastname: Lansigan
authors.forename: Iris
authors.initials: I
authors.lastname: Isufi
authors.forename: Clement E
authors.initials: CE
authors.lastname: Tagoe
mesh_terms: D051722:ADAM Proteins / Q000502:physiology*; D000071120:ADAMTS13 Protein; D000743:Anemia, Hemolytic / Q000175:diagnosis / Q000209:etiology* / Q000503:physiopathology; D003937:Diagnosis, Differential; D006801:Humans; D008180:Lupus Erythematosus, Systemic / Q000150:complications* / Q000503:physiopathology; D011379:Prognosis; D011697:Purpura, Thrombotic Thrombocytopenic / Q000175:diagnosis / Q000209:etiology* / Q000503:physiopathology; D057049:Thrombotic Microangiopathies / Q000175:diagnosis / Q000209:etiology / Q000503:physiopathology
chemical_list: D051722:ADAM Proteins; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human