A patient with SLE-associated thrombotic microangiopathy and non-neutralizing antibodies against ADAMTS13.
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.
Knecht
Margrith E
ME
Department of Internal Medicine, University Hospital Basel, Basel, Switzerland. margrith.knecht@gzf.ch
Mayr
Michael
M
Ferrari
Silvia
S
Scheiflinger
Friedrich
F
Trendelenburg
Marten
M
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*
D001323:Autoantibodies; D051722:ADAM Proteins; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human