title: Glucocorticoid and cyclosporine refractory adult onset Still's disease successfully treated with tocilizumab.
abstract: We report a 29-year-old Japanese woman with disseminated intravascular coagulation (DIC) and adult onset Still's disease (AOSD). Her disease was refractory to high-dose glucocorticoids, two courses of steroid pulse therapy, and addition of cyclosporine (3.5 mg/kg/day). The serum interleukin-6 level was markedly elevated. Therefore, we administered an anti-interleukin-6 receptor antibody (tocilizumab, 8 mg/kg fortnightly), which dramatically improved her symptoms and the levels of acute-phase proteins. In addition, rapid tapering of the glucocorticoid dose was possible. Four months later, she was maintained on tocilizumab infusion once a month with low-dose steroid therapy. Cyclosporine is one of the first-line immunosuppressants for AOSD, especially when associated with DIC, hepatic failure, or hemophagocytic syndrome. In patients with cyclosporine-resistant AOSD, tocilizumab may be another useful option.
authors.affiliation: Division of Rheumatology and Clinical Immunology, Jichi Medical University, Yakushiji 3311-1, Shimotsuke-shi, Tochigi 329-0498, Japan.
authors.forename: Kazuko
authors.initials: K
authors.lastname: Matsumoto
authors.forename: Takao
authors.initials: T
authors.lastname: Nagashima
authors.forename: Shino
authors.initials: S
authors.lastname: Takatori
authors.forename: Yuta
authors.initials: Y
authors.lastname: Kawahara
authors.forename: Masaki
authors.initials: M
authors.lastname: Yagi
authors.forename: Masahiro
authors.initials: M
authors.lastname: Iwamoto
authors.forename: Hitoaki
authors.initials: H
authors.lastname: Okazaki
authors.forename: Seiji
authors.initials: S
authors.lastname: Minota
mesh_terms: D000328:Adult; D000911:Antibodies, Monoclonal / Q000494:pharmacology*; D061067:Antibodies, Monoclonal, Humanized; D016572:Cyclosporine / Q000494:pharmacology*; D004211:Disseminated Intravascular Coagulation / Q000175:diagnosis* / Q000188:drug therapy; D005260:Female; D005938:Glucocorticoids / Q000494:pharmacology*; D006801:Humans; D007166:Immunosuppressive Agents / Q000494:pharmacology; D015850:Interleukin-6 / Q000378:metabolism; D016706:Still's Disease, Adult-Onset / Q000188:drug therapy*; D013997:Time Factors
chemical_list: D000911:Antibodies, Monoclonal; D061067:Antibodies, Monoclonal, Humanized; D005938:Glucocorticoids; D007166:Immunosuppressive Agents; D015850:Interleukin-6; D016572:Cyclosporine; C502936:tocilizumab