title: Two cases of mixed connective tissue disease complicated with thrombotic thrombocytopenic purpura.
abstract: Thrombotic thrombocytopenic purpura (TTP) is a rare complication of connective tissue diseases. Here, we describe 2 patients with mixed connective tissue disease (MCTD) who had pericardial effusion and TTP. They had moderately low to normal ADAMTS13 activity and no ADAMTS13 neutralizing antibodies. Both patients responded poorly to plasma exchange. One patient died due to multiple organ failure and the other due to infection. Although rare, TTP is an important complication that leads to a poor prognosis in MCTD patients.
authors.affiliation: Division of Rheumatology, Ohta-Nishinouchi Hospital, Koriyama, Japan. azsuzuki@fmu.ac.jp
authors.forename: Eiji
authors.initials: E
authors.lastname: Suzuki
authors.forename: Takashi
authors.initials: T
authors.lastname: Kanno
authors.forename: Tomoyuki
authors.initials: T
authors.lastname: Asano
authors.forename: Akito
authors.initials: A
authors.lastname: Tsutsumi
authors.forename: Hiroko
authors.initials: H
authors.lastname: Kobayashi
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Watanabe
authors.forename: Hiromasa
authors.initials: H
authors.lastname: Ohira
mesh_terms: D051722:ADAM Proteins / Q000032:analysis; D000071120:ADAMTS13 Protein; D005260:Female; D006801:Humans; D008875:Middle Aged; D008947:Mixed Connective Tissue Disease / Q000150:complications*; D010951:Plasma Exchange; D011697:Purpura, Thrombotic Thrombocytopenic / Q000209:etiology* / Q000628:therapy; D057049:Thrombotic Microangiopathies / Q000209:etiology
chemical_list: D051722:ADAM Proteins; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human