title: A case of mixed connective tissue disease complicated with thrombotic thrombocytopenic purpura.
abstract: Thrombotic thrombocytopenic purpura (TTP) is a rare complication of mixed connective tissue disease (MCTD). In this report, we describe the case of a 73-year-old Japanese woman with MCTD who developed fever, thrombocytopenia, and microangiopathic hemolytic anemia and was diagnosed with MCTD together with TTP. The activity of von Willebrand factor (vWF) cleaving metalloprotease ADAMTS13 was low and considered to have contributed to the disease activity of TTP. The patient died despite intensive treatment of plasma exchange (PEX) and steroid pulse therapy. Autopsy results revealed that the kidneys had platelet and fibrin thrombi, which occluded capillaries and arterioles. These findings were compatible with TTP and the decreased activity of ADAMTS13 was considered to be associated with the disease activity of TTP.
authors.affiliation: Division of Clinical Nephrology and Rheumatology, Niigata University Graduate School of Medical and Dental Science, Asahimachi-Dori, Niigata City, Japan. kurodat@med.niigata-u.ac.jp
authors.forename: Takeshi
authors.initials: T
authors.lastname: Kuroda
authors.forename: Kouki
authors.initials: K
authors.lastname: Matsuyama
authors.forename: Takeshi
authors.initials: T
authors.lastname: Nakatsue
authors.forename: Syuuichi
authors.initials: S
authors.lastname: Murakami
authors.forename: Hisashi
authors.initials: H
authors.lastname: Hasegawa
authors.forename: Hideaki
authors.initials: H
authors.lastname: Nakayama
authors.forename: Minoru
authors.initials: M
authors.lastname: Sakatsume
authors.forename: Mitsuhiro
authors.initials: M
authors.lastname: Ueno
authors.forename: Masaaki
authors.initials: M
authors.lastname: Nakano
authors.forename: Fumitake
authors.initials: F
authors.lastname: Gejyo
mesh_terms: D051722:ADAM Proteins / Q000097:blood / Q000172:deficiency; D000071120:ADAMTS13 Protein; D000368:Aged; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D008947:Mixed Connective Tissue Disease / Q000097:blood / Q000150:complications*; D010951:Plasma Exchange; D011697:Purpura, Thrombotic Thrombocytopenic / Q000097:blood / Q000150:complications*
chemical_list: D051722:ADAM Proteins; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human