Successful tocilizumab therapy in seven patients with refractory adult-onset Still's disease
Ei BannaiHiroyuki YamashitaShunta KanekoYo UedaTakashi OzakiHaruka TsuchiyaYuko TakahashiHiroshi KanekoToshikazu KanoAkio Mimori
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Journal Article Successful tocilizumab therapy in seven patients with refractory adult-onset Still's disease Get access Ei Bannai, Ei Bannai Division of Rheumatic Diseases, National Center for Global Health and Medicine, Shinjuku-ku, Tokyo, Japan Search for other works by this author on: Oxford Academic Google Scholar Hiroyuki Yamashita, Hiroyuki Yamashita Division of Rheumatic Diseases, National Center for Global Health and Medicine, Shinjuku-ku, Tokyo, Japan Correspondence to: Hiroyuki Yamashita, Division of Rheumatic Diseases, National Center for Global Health and Medicine, 1-21-1 Toyama, Shinjuku, Tokyo 162-8655, Japan. Tel: + 81-3-3202-7181. Fax: + 81-3-3207-1038. E-mail: hiroyuki_yjp2005@yahoo.co.jp Search for other works by this author on: Oxford Academic Google Scholar Shunta Kaneko, Shunta Kaneko Division of Rheumatic Diseases, National Center for Global Health and Medicine, Shinjuku-ku, Tokyo, Japan Search for other works by this author on: Oxford Academic Google Scholar Yo Ueda, Yo Ueda Division of Rheumatic Diseases, National Center for Global Health and Medicine, Shinjuku-ku, Tokyo, Japan Search for other works by this author on: Oxford Academic Google Scholar Takashi Ozaki, Takashi Ozaki Division of Rheumatic Diseases, National Center for Global Health and Medicine, Shinjuku-ku, Tokyo, Japan Search for other works by this author on: Oxford Academic Google Scholar Haruka Tsuchiya, Haruka Tsuchiya Division of Rheumatic Diseases, National Center for Global Health and Medicine, Shinjuku-ku, Tokyo, Japan Search for other works by this author on: Oxford Academic Google Scholar Yuko Takahashi, Yuko Takahashi Division of Rheumatic Diseases, National Center for Global Health and Medicine, Shinjuku-ku, Tokyo, Japan Search for other works by this author on: Oxford Academic Google Scholar Hiroshi Kaneko, Hiroshi Kaneko Division of Rheumatic Diseases, National Center for Global Health and Medicine, Shinjuku-ku, Tokyo, Japan Search for other works by this author on: Oxford Academic Google Scholar Toshikazu Kano, Toshikazu Kano Division of Rheumatic Diseases, National Center for Global Health and Medicine, Shinjuku-ku, Tokyo, Japan Search for other works by this author on: Oxford Academic Google Scholar Akio Mimori Akio Mimori Division of Rheumatic Diseases, National Center for Global Health and Medicine, Shinjuku-ku, Tokyo, Japan Search for other works by this author on: Oxford Academic Google Scholar Modern Rheumatology, Volume 26, Issue 2, 3 March 2016, Pages 297–301, https://doi.org/10.3109/14397595.2014.899178 Published: 03 March 2016 Article history Received: 06 August 2013 Accepted: 24 November 2013 Published: 03 March 2016Keywords:
Tocilizumab
Adult-onset Still's disease
Macrophage Activation Syndrome
Refractory (planetary science)
Antirheumatic drugs
Abstract We report a case of 50-year-old female patient with adult-onset Still’s disease (AOSD) complicated by macrophage-activation syndrome (MAS). After initial control of the disease with high-dose parenteral corticosteroids, tocilizumab (TCZ) therapy aided in maintaining the remission with rapid tapering of steroid dose. TCZ may be useful for MAS complicating AOSD.
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Tocilizumab (TCZ) is the first FDA- approved treatment for systemic juvenile idiopathic arthritis (sJIA). We report 3 cases of cytopenias in children with sJIA treated with TCZ. Two of the children who developed significant cytopenias shortly after initiation of TCZ had a history of macrophage activation syndrome. We raise the possibility that patients with a tendency towards MAS have an increased risk of developing cytopenias when treated with tocilizumab.
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Adult-onset Still's disease (AOSD) usually affects young adults. Some cases of elderly-onset Still's disease (EOSD) have been reported, but its clinical features are unclear. We herein report a 74-year-old woman who developed AOSD with macrophage activation syndrome (MAS). We also reviewed 24 previous EOSD cases in patients over 70 years old and compared the findings with overall AOSD. While the clinical features were similar between the two, including the presence of MAS, disseminated intravascular coagulation was more frequent in EOSD than in AOSD. Furthermore, despite a similar frequency of glucocorticoid use, immunosuppressants and biologics were less frequently administered in EOSD than in AOSD. This report highlights the fact that typical AOSD can develop in elderly patients with some characteristic features.
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Response to: 'Tocilizumab in patients with adult-onset Still's disease refractory to glucocorticoid treatment' by LeeWe would like to thank Dr Lee 1 for his interest in our paper 2 and for his comments providing futuristic insights into the management of adult-onset Still's disease.4][5] Although the safety of biological agents including tocilizumab have been shown in patients with rheumatoid arthritis, they are more expensive than conventional DMARDs, and the long-term safety of their use in patients with adult-onset Still's disease is still unknown.Some of patients in our trial had a history of not responding to DMARDs such as methotrexate or ciclosporine, but we did not collect precise information about patients' previous treatment other than glucocorticoids use.Our trial was a first step, aimed at proving the efficacy of antiinterleukin-6 treatment by a high-levelled evidence rather than case reports.As Dr Lee mentioned, further randomised studies are warranted to determine the optimal management of adultonset Still's disease, although the rarity and occasional fatal severity of adult-onset Still's disease would hinder determining appropriate endpoints and recruiting active patients who are refractory to glucocorticoids but can tolerate control treatment including placebo or conventional DMARDs.The next step will need worldwide cooperation to establish the optimal management of adult-onset Still's in clinical studies with a proper design and sample size.
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We report the case of a young man who presented with high-spiking fever, erosive arthritis, and generalized lymphadenopathy. He was diagnosed as adult-onset Still's disease (AOSD), and his clinical course was complicated by reactive macrophage activation syndrome (MAS). The sudden precipitous drop in leukocyte and platelet counts proved a valuable clue to the diagnosis of MAS in the setting of AOSD. The new American College of Rheumatology/European League Against Rheumatism classification criteria for reactive MAS complicating systemic juvenile idiopathic arthritis were applied in this patient. When MAS is associated with AOSD, treatment could be challenging. We managed this patient successfully with a combination of systemic corticosteroids and cyclosporine in the acute phase.
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We describe a case of adult-onset Still’s disease (AOSD) with secondary macrophage activation syndrome. AOSD is a rare disease with unknown origin. Rarely it’s complicated by secondary macrophage activation syndrome, which is a life-threatening disorder. Because the diagnosis of AOSD is essential for exclusion and complications are life-threatening, it is mandatory for a prompt and extensive diagnostic workup and treatment.
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Macrophage Activation Syndrome
Adult-onset Still's disease
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We report a 57-year-old female case of intractable adult-onset Still's disease (AOSD). Initial high-dose prednisolone therapy was ineffective, and macrophage-activation syndrome (MAS) manifested after one session of additional tocilizumab therapy. After successful treatment for MAS with lipo-dexamethasone and cyclosporin, tocilizumab therapy aided in the rapid reduction of the therapeutic steroid dose. Tocilizumab may be useful for maintenance therapy for AOSD, although its efficacy is unclear for the highly active phase of the disease.
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Adult-onset Still's disease
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Antirheumatic drugs
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Cytokine Release Syndrome
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Systemic juvenile idiopathic arthritis (sjia) is a chronic inflammatory disease characterized by prolonged systemic and synovial inflammation. Life-threatening complication of sjia is the macrophage activation syndrome (MAS). Tocilizumab (TCZ) is highly effective in children with sjia. However, MAS remains under the treatment of TCZ.
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