title: A patient with SLE-associated thrombotic microangiopathy and non-neutralizing antibodies against ADAMTS13.
abstract: In this case report, we describe for the first time a patient with thrombotic thrombocytopaenic purpura (TTP) accompanying highly active systemic lupus erythematosus (SLE) that was associated with non-neutralizing antibodies against the plasma metalloprotease ADAMTS13. Those non-neutralizing antibodies could have been the cause of ADAMTS13 depletion and consecutive TTP. More extensive analyses are required to determine the prevalence as well as the clinical relevance of non-neutralizing antibodies against ADAMTS13 in SLE patients.
authors.affiliation: Department of Internal Medicine, University Hospital Basel, Basel, Switzerland. margrith.knecht@gzf.ch
authors.forename: Margrith E
authors.initials: ME
authors.lastname: Knecht
authors.forename: Michael
authors.initials: M
authors.lastname: Mayr
authors.forename: Silvia
authors.initials: S
authors.lastname: Ferrari
authors.forename: Friedrich
authors.initials: F
authors.lastname: Scheiflinger
authors.forename: Marten
authors.initials: M
authors.lastname: Trendelenburg
mesh_terms: D051722:ADAM Proteins / Q000276:immunology*; D000071120:ADAMTS13 Protein; D000328:Adult; D001323:Autoantibodies / Q000097:blood*; D005260:Female; D006801:Humans; D008180:Lupus Erythematosus, Systemic / Q000150:complications*; D057049:Thrombotic Microangiopathies / Q000209:etiology*
chemical_list: D001323:Autoantibodies; D051722:ADAM Proteins; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human