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Efficacy of abatacept for IgG4-related disease over 8 months
  1. Motohisa Yamamoto1,
  2. Hiroki Takahashi1,
  3. Kenichi Takano2,
  4. Yui Shimizu1,
  5. Nodoka Sakurai1,
  6. Chisako Suzuki1,
  7. Yasuyoshi Naishiro1,
  8. Hidetaka Yajima1,3,
  9. Teruhito Awakawa3,
  10. Tetsuo Himi2,
  11. Hiroshi Nakase1
  1. 1 Department of Gastroenterology, Rheumatology and Clinical Immunology, Sapporo Medical University School of Medicine, Sapporo, Hokkaido, Japan
  2. 2 Department of Otolaryngology, Sapporo Medical University School of Medicine, Sapporo, Hokkaido, Japan
  3. 3 Department of Internal Medicine, Sapporo Dohkohkai Hospital, Sapporo, Hokkaido, Japan
  1. Correspondence to Dr Motohisa Yamamoto, Department of Gastroenterology, Rheumatology and Clinical Immunology, Sapporo Medical University School of Medicine, South 1- West 16, Chuo-ku, Sapporo, Hokkaido 0608543, Japan; mocha{at}cocoa.plala.or.jp

https://doi.org/10.1136/annrheumdis-2016-209368

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    It was recently pointed out that rituximab (RTX) is effective against IgG4-related disease (IgG4-RD).1 We found that RTX is effective as an induction therapy for IgG4-RD in the short term, but that it is necessary to repeat the prescription of RTX. Furthermore, we had a patient who presented with resistance to RTX.2 After obtaining informed consent, we started the patient on abatacept (ABT) treatment. We have followed the patient for 8 months, and the patient presented with good response to ABT. This is the first report of the efficacy of ABT against IgG4-RD.

    The patient was a 65-year-old Japanese woman who presented with IgG4-related dacryoadenitis and sialadenitis, and autoimmune pancreatitis. A submandibular gland biopsy specimen showed prominent infiltration of IgG4-bearing plasma cells (the ratio of IgG4/IgG-positive cells: 40%) with fibrosis and germinal centres (figure 1A, B). We diagnosed this case as IgG4-RD.3 She was initially treated with 40 mg/day of prednisolone, but several relapses occurred with tapering the dose to 10 mg/day. We administered ciclosporin A and mizoribine with steroid, …

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    Footnotes

    • Contributors All members were involved in the discussion of her treatment.

    • Funding This work was supported by the Research on Measures for Intractable Diseases Project matching fund subsidy from the Ministry of Health, Labour, and Welfare, Japan and the Japan Agency for Medical Research and Development.

    • Competing interests None declared.

    • Patient consent Obtained.

    • Ethics approval The protocol was approved by Sapporo Medical University Hospital Institutional Review Board subject to applicable laws and regulations and ethical principles consistent with the Declaration of Helsinki.

    • Provenance and peer review Not commissioned; externally peer reviewed.