A case report of a patient with refractory adult-onset Still’s disease who was successfully treated with tocilizumab over 6 years
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Abstract:
Interleukin-6 overproduction is pathologically involved in adult onset Still's disease (AOSD). We successfully treated a man with refractory AOSD utilizing tocilizumab. Tocilizumab was discontinued after 15 doses due to intestinal bleeding, but the efficacy was sustained over 21 months. Tocilizumab was readministered safely upon recurrence and showed similar efficacy over 6 years. Corticosteroid and NSAIDs could be discontinued and intestinal bleeding was no more observed. Tocilizumab can be a therapeutic option for AOSD.Keywords:
Tocilizumab
Refractory (planetary science)
Adult-onset Still's disease
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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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 2016
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Macrophage Activation Syndrome
Refractory (planetary science)
Antirheumatic drugs
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Takayasu arteritis (TA) is an idiopathic inflammatory vasculitis mostly affecting young females in the second or third decades of life. Corticosteroids and conventional immunosuppressants remain the mainstay of treatment for TA, but refractory cases are dealt with biological agents. The high cost and longer duration of therapy are issues of concern. Here, we report a case of a patient with refractory TA who underwent successful treatment with tocilizumab, a humanized monoclonal antibody against interleukin-6 receptor.
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Refractory (planetary science)
Takayasu Arteritis
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Arteritis
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Journal Article A case report of a patient with refractory adult-onset Still's disease who was successfully treated with tocilizumab over 6 years Get access Hideko Nakahara, Hideko Nakahara Laboratory of Immune Regulation, Graduate School of Frontier Biosciences, Osaka University, 1-3 Yamada-oka, Suita, Osaka 565-0871, JapanNTT West Osaka Hospital, 2-6-40 Karasugatsuji, Tennoji-ku, Osaka, Osaka 543-8922, Japan Search for other works by this author on: Oxford Academic Google Scholar Toru Mima, Toru Mima Laboratory of Immune Regulation, Graduate School of Frontier Biosciences, Osaka University, 1-3 Yamada-oka, Suita, Osaka 565-0871, Japan Search for other works by this author on: Oxford Academic Google Scholar Naoko Yoshio-Hoshino, Naoko Yoshio-Hoshino Laboratory of Immune Regulation, Graduate School of Frontier Biosciences, Osaka University, 1-3 Yamada-oka, Suita, Osaka 565-0871, Japan Search for other works by this author on: Oxford Academic Google Scholar Masato Matsushita, Masato Matsushita Division of Rheumatology, National Hospital Organization, Osaka Minami Medical Center, 2-1 Kidohigashi-cho, Kawachinagano, Osaka 586-8521, Japan Search for other works by this author on: Oxford Academic Google Scholar Jun Hashimoto, Jun Hashimoto Department of Orthopaedics, Osaka University Graduate School of Medicine, 2-2 Yamada-oka, Suita, Osaka 565-0871, Japan Search for other works by this author on: Oxford Academic Google Scholar Norihiro Nishimoto Norihiro Nishimoto Laboratory of Immune Regulation, Graduate School of Frontier Biosciences, Osaka University, 1-3 Yamada-oka, Suita, Osaka 565-0871, Japan Correspondence to: Norihiro Nishimoto, Laboratory of Immune Regulation, Graduate School of Frontier Biosciences, Osaka University, 1-3 Yamada-oka, Suita, Osaka 565-0871, Japan e-mail: norihiro@fbs.osaka-u.ac.jp Search for other works by this author on: Oxford Academic Google Scholar Modern Rheumatology, Volume 19, Issue 1, 1 February 2009, Pages 69–72, https://doi.org/10.3109/s10165-008-0116-2 Published: 01 February 2009 Article history Received: 25 April 2008 Accepted: 22 July 2008 Published: 01 February 2009
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Abstract Objectives Macrophage activation syndrome (MAS) developed under tocilizumab treatment poses a diagnostic challenge. This study aims to demonstrate the frequency and the clinical features of MAS developed in patients with adult-onset Still’s disease (AOSD) receiving tocilizumab. Methods The consecutive AOSD patients treated with tocilizumab in our institution from April 2008 to March 2020 were studied. The frequency of clinically diagnosed MAS during tocilizumab treatment, their conformity to the several criteria relevant for MAS, and laboratory characteristics compared to AOSD flare were investigated. Results Of the 20 AOSD patients treated with tocilizumab, six developed clinically diagnosed MAS, four immediately after starting tocilizumab and two after long-term treatment. Some of them had already met the MAS criteria before starting tocilizumab. At MAS diagnosis, although some did not meet the MAS criteria due to lack of fever and/or the lower ferritin levels, all consistently showed sharp increases in ferritin along with marked abnormal changes in two or more different markers of organ damage, unlike the AOSD flares. Conclusion MAS is not a rare complication in AOSD patients receiving tocilizumab. The clinical similarities between systemic AOSD and MAS, and substantial alterations in MAS features by inhibition of interleukin-6 signaling may limit the utility of the existing diagnostic/classification criteria in diagnosing MAS under tocilizumab treatment. The emergence of abnormalities in MAS-related organ damage markers with a rapid elevation of ferritin should be considered as MAS development in AOSD patients receiving tocilizumab even if the patients are afebrile or have relatively low ferritin levels.
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Macrophage Activation Syndrome
Adult-onset Still's disease
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Tocilizumab
Adult-onset Still's disease
Refractory (planetary science)
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There have been many previously reported cases of adult-onset Still's disease (AOSD) which were successfully treated with tocilizumab (TCZ). However, the efficacy and safety of TCZ therapy for AOSD-associated macrophage activation syndrome (MAS), and the optimal duration of TCZ therapy, remain unclear. We herein report two cases of refractory AOSD, one of which was associated with MAS. These two patients were treated with TCZ, and the withdrawal of TCZ was planned according to the serum interleukin-6 level, which resulted in TCZ-free remission.
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Adult-onset Still's disease
Refractory (planetary science)
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Macrophage Activation Syndrome
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Results Forty-two SJIA patients, 131 blood samplings were included in this study. Seventeen patients (40%) were treated with tocilizumab during the study. Serum IL-6 levels in patients without tocilizumab treatment significantly elevated in active disease with systemic features and arthritis [median (IQR) = 101.8 (303.2) pg/mL] when compared to active disease with only arthritis [median (IQR) = 4.5 (23) pg/mL], and remission on medication [median (IQR) = 1.5 (0.55) pg/mL], whereas serum IL-6 levels in patients with tocilizumab treatment were not different between groups but there were significantly different when compared to healthy children (p < 0.05). In addition, the correlation between serum IL-6 levels and JADAS-71 in patients without tocilizumab treatment (r = 0.71, p < 0.001) was stronger than patients with tocilizumab treatment (r = 0.42, p = 0.01). Serum sIL-6R levels in SJIA patients with and without tocilizumab treatment were significantly higher when compared to healthy children (p < 0.05). Interestingly, in patients with tocilizumab treatment, serum sIL-6R levels were extremely higher [median (IQR) = 1,110.3 (840.2) ng/mL] than patients without tocilizumab treatment [median (IQR) = 94.2 (82.7) ng/mL]. Conclusion The correlation between serum IL-6 levels and disease activity in patients without tocilizumab treatment was stronger than patients with tocilizumab treatment. In addition, serum sIL-6R levels in patients with tocilizumab treatment were extremely higher than patients without tocilizumab treatment.
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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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Macrophage Activation Syndrome
Adult-onset Still's disease
Prednisolone
Antirheumatic drugs
Combination therapy
Cytokine Release Syndrome
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