title: Goodpasture's syndrome associated with thrombotic thrombocytopenic purpura secondary to an ADAMTS-13 deficit.
abstract: A 27-year-old man was hospitalized for acute kidney injury associated with antiglomerular basement membrane antibodies (anti-GBM). He underwent immunosuppression and plasma exchange therapy, without recovery of renal function. Later on, he was again admitted to the hospital with seizures. Evidence of microangiopathic hemolytic anemia, with schistocytes in peripheral blood, was present, as well as a persistent low platelet count and activity of von Willebrand factor from adherence to protease (ADAMTS-13) less than 1 %. The presence of IgG antibodies against ADAMTS-13 was documented, leading to a diagnosis of thrombotic thrombocytopenic purpura (TTP) in the context of Goodpasture's syndrome. The TTP was treated with rituximab and plasmapheresis with a good response. We conclude that early measurement of ADAMTS-13 activity dictated the most appropriate therapy and achieved excellent results in this patient.
authors.affiliation: Department of Nephrology, University Hospital La Paz, IDIPaz, Paseo de la Castellana 261, 28046, Madrid, Spain, mcvega.hulp@salud.madrid.org.
authors.forename: Cristina
authors.initials: C
authors.lastname: Vega-Cabrera
authors.forename: Gloria
authors.initials: G
authors.lastname: Del Peso
authors.forename: Auxiliadora
authors.initials: A
authors.lastname: Bajo
authors.forename: M-Luz
authors.initials: ML
authors.lastname: Picazo
authors.forename: Begoña
authors.initials: B
authors.lastname: Rivas-Becerra
authors.forename: Ana-Lidia
authors.initials: AL
authors.lastname: Benitez
authors.forename: Jorge Martínez
authors.initials: JM
authors.lastname: Ara
authors.forename: Teresa
authors.initials: T
authors.lastname: Olea
authors.forename: Rafael
authors.initials: R
authors.lastname: Selgas
mesh_terms: D051722:ADAM Proteins / Q000172:deficiency* / Q000276:immunology; D000071120:ADAMTS13 Protein; D000328:Adult; D019867:Anti-Glomerular Basement Membrane Disease / Q000150:complications* / Q000473:pathology; D058846:Antibodies, Monoclonal, Murine-Derived / Q000627:therapeutic use; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood; D007155:Immunologic Factors / Q000627:therapeutic use; D008297:Male; D010956:Plasmapheresis; D010976:Platelet Count; D011697:Purpura, Thrombotic Thrombocytopenic / Q000097:blood* / Q000150:complications / Q000628:therapy; D000069283:Rituximab
chemical_list: D058846:Antibodies, Monoclonal, Murine-Derived; D007074:Immunoglobulin G; D007155:Immunologic Factors; D000069283:Rituximab; D051722:ADAM Proteins; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human