title: Plasma ADAMTS13, von Willebrand Factor (VWF), and VWF Propeptide Profiles in Patients With Connective Tissue Diseases and Antiphospholipid Syndrome.
abstract: Thrombotic thrombocytopenic purpura (TTP) frequently develops in patients with connective tissue diseases (CTDs). ADAMTS13 and von Willebrand factor (VWF) are closely related to the onset of TTP. We investigated the roles of ADAMTS13 and VWF in thrombotic events of patients with CTD. ADAMTS13 activity and VWF and VWF propeptide (VWFpp) levels in CTD, primary antiphospholipid antibody syndrome (pAPS), and controls were measured to examine their relationship with thrombosis. ADAMTS13 activity levels were significantly low in the patients with CTD but not in the patients with pAPS. No significant difference in the ADAMTS13 activity levels among the various CTD subgroups was found. The levels of VWF and VWFpp were significantly elevated in the patients with pAPS and CTD compared with that of control groups. Eleven patients with CTD developed TTP, and their ADAMTS13 activity levels were significantly lower than patients having CTD without TTP. However, the ADAMTS13 activity levels showed no difference between the patients having CTD with and without thrombotic events. The VWF antigen levels were significantly high in the patients having CTD with TTP. There were no significant differences in the VWF levels of the patients having CTD with TTP and thrombosis. The VWFpp levels were significantly high in the patients having CTD with TTP and thrombosis. The VWF and VWFpp levels were significantly high in the patients with pAPS. Decreased ADAMTS13 activity and elevated VWF and VWFpp levels were observed in patients with CTD. These abnormalities in patients with CTD may represent the increased risk of thrombosis in CTD.
authors.affiliation: 1 Department of Dermatology, Mie University Graduate School of Medicine, Mie, Tsu, Japan.
authors.forename: Koji
authors.initials: K
authors.lastname: Habe
authors.affiliation: 2 Department of Molecular and Laboratory Medicine, Mie University Graduate School of Medicine, Tsu, Japan.
authors.forename: Hideo
authors.initials: H
authors.lastname: Wada
authors.affiliation: 3 Blood Transfusion Service, Mie University Hospital, Mie, Tsu, Japan.
authors.forename: Takeshi
authors.initials: T
authors.lastname: Matsumoto
authors.affiliation: 3 Blood Transfusion Service, Mie University Hospital, Mie, Tsu, Japan.
authors.forename: Kohshi
authors.initials: K
authors.lastname: Ohishi
authors.affiliation: 4 Central laboratory, Mie University Hospital, Tsu, Japan.
authors.forename: Makoto
authors.initials: M
authors.lastname: Ikejiri
authors.affiliation: 1 Department of Dermatology, Mie University Graduate School of Medicine, Mie, Tsu, Japan.
authors.forename: Kenshiro
authors.initials: K
authors.lastname: Tsuda
authors.affiliation: 1 Department of Dermatology, Mie University Graduate School of Medicine, Mie, Tsu, Japan.
authors.forename: Makoto
authors.initials: M
authors.lastname: Kondo
authors.affiliation: 5 Department of Obstetrics and Gynecology, Mie University Graduate School of Medicine, Mie, Tsu, Japan.
authors.forename: Yuki
authors.initials: Y
authors.lastname: Kamimoto
authors.affiliation: 5 Department of Obstetrics and Gynecology, Mie University Graduate School of Medicine, Mie, Tsu, Japan.
authors.forename: Tomoaki
authors.initials: T
authors.lastname: Ikeda
authors.affiliation: 6 Department of Hematology and Oncology, Mie University Graduate School of Medicine, Mie, Tsu, Japan.
authors.forename: Naoyuki
authors.initials: N
authors.lastname: Katayama
authors.affiliation: 1 Department of Dermatology, Mie University Graduate School of Medicine, Mie, Tsu, Japan.
authors.forename: Hitoshi
authors.initials: H
authors.lastname: Mizutani
mesh_terms: D000071120:ADAMTS13 Protein / Q000097:blood*; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D016736:Antiphospholipid Syndrome / Q000097:blood / Q000150:complications*; D016022:Case-Control Studies; D003240:Connective Tissue Diseases / Q000097:blood / Q000150:complications*; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D011697:Purpura, Thrombotic Thrombocytopenic; D013927:Thrombosis / Q000209:etiology; D055815:Young Adult; D014841:von Willebrand Factor / Q000032:analysis*
chemical_list: D014841:von Willebrand Factor; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human
keywords: ADAMTS13; VWF propeptide; antiphospholipid syndrome; connective tissue disease; von Willebrand factor (VWF)