title: [Thrombotic thrombocytopenic purpura in patients with systemic lupus erythematosus].
abstract: Although not common, thrombotic thrombocytopenic purpura (TTP) is one of the most serious complications in patients with systemic lupus erythematosus (SLE). This study aimed to characterize the association between SLE and TTP by examining clinical features and treatment outcomes. We evaluated eight patients diagnosed over two years in our hospital. The mean disease duration of SLE until TTP onset was 13.2 years. One patient had a simultaneous diagnosis of SLE and TTP. CNS lupus and lupus nephritis were involved in 6 patients, respectively. The mean value of the SLE disease activity index was 40.5. Five out of 7 patients who have been examined the von Willebrand factor cleaving protease showed moderately decreased activity. Seven out of the 8 patients were treated with plasmapheresis, and 7 received immunosuppressive therapy including cyclophosphamide and rituximab. Seven patients recovered, but one died from an exacerbation of the disease. These data indicated that plasmapheresis and immunosuppressive therapy improved prognosis of TTP associated with SLE.
authors.affiliation: Department of Internal Medicine and Rheumatology, Clinical Research Institute, National Hospital Organization, Kyushu Medical Center, Japan.
authors.forename: Tomoya
authors.initials: T
authors.lastname: Miyamura
authors.forename: Hideyuki
authors.initials: H
authors.lastname: Watanabe
authors.forename: Soichiro
authors.initials: S
authors.lastname: Takahama
authors.forename: Koshiro
authors.initials: K
authors.lastname: Sonomoto
authors.forename: Masataka
authors.initials: M
authors.lastname: Nakamura
authors.forename: Hitosi
authors.initials: H
authors.lastname: Ando
authors.forename: Rumi
authors.initials: R
authors.lastname: Minami
authors.forename: Masahiro
authors.initials: M
authors.lastname: Yamamoto
authors.forename: Eiichi
authors.initials: E
authors.lastname: Suematsu
mesh_terms: D000328:Adult; D000911:Antibodies, Monoclonal / Q000627:therapeutic use; D058846:Antibodies, Monoclonal, Murine-Derived; D005260:Female; D006801:Humans; D007155:Immunologic Factors / Q000627:therapeutic use; D008180:Lupus Erythematosus, Systemic / Q000150:complications*; D008297:Male; D008875:Middle Aged; D010956:Plasmapheresis; D011697:Purpura, Thrombotic Thrombocytopenic / Q000150:complications* / Q000628:therapy; D000069283:Rituximab
chemical_list: D000911:Antibodies, Monoclonal; D058846:Antibodies, Monoclonal, Murine-Derived; D007155:Immunologic Factors; D000069283:Rituximab