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A case of MPO- and PR3-ANCA-positive hypertrophic cranial pachymeningitis with elevated serum IgG4

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Modern Rheumatology

Abstract

We report a case of orbital tumor and hypertrophic cranial pachymeningitis in a 64-year-old woman, who was initially suspected to have IgG4-related disease because of an elevated level of serum IgG4 at onset. However, her condition was resistant to glucocorticoid therapy, and additional cyclophosphamide was necessary to control the disease activity. Additional features included elevated levels of serum myeloperoxidase (MPO) and proteinase-3-anti-neutrophil cytoplasmic antibody (PR3-ANCA), and biopsy specimens from the orbital mass revealed very few infiltrating IgG4-positive cells. Instead, rupture of the elastic layer of the arterial walls with neovascularization and a small number of giant cells were observed. Considering these findings and the clinical course, the disease was considered more likely to be ANCA-associated pachymeningitis with elevation of the serum IgG4 level.

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Acknowledgments

The authors are grateful to Dr. Takako Saeki, MD, PhD, Nagaoka Red Cross Hospital, Niigata, Japan, for valuable suggestions concerning the manuscript.

Conflict of interest

None.

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Authors and Affiliations

  1. Division of Clinical Nephrology and Rheumatology, Department of Medicine II, Niigata University Graduate School of Medical and Dental Sciences, 1-757 Asahimachi-dori, Chuo-ku, Niigata, Niigata, 951-8510, Japan

    Akira Iguchi, Yoko Wada, Daisuke Kobayashi, Hiroe Sato, Tokuhide Oyama, Takeshi Nakatsue, Shuichi Murakami, Takeshi Kuroda, Masaaki Nakano & Ichiei Narita

  2. Division of Ophthalmology and Visual Sciences, Niigata University Graduate School of Medical and Dental Sciences, Niigata, Japan

    Akira Iguchi, Yoko Wada, Daisuke Kobayashi, Hiroe Sato, Tokuhide Oyama, Takeshi Nakatsue, Shuichi Murakami, Takeshi Kuroda, Masaaki Nakano & Ichiei Narita

  3. Health Administration Center of Niigata University, Niigata, Japan

    Akira Iguchi, Yoko Wada, Daisuke Kobayashi, Hiroe Sato, Tokuhide Oyama, Takeshi Nakatsue, Shuichi Murakami, Takeshi Kuroda, Masaaki Nakano & Ichiei Narita

  4. Department of Medical Technology, Faculty of Medicine, School of Health Sciences, Niigata University, Niigata, Japan

    Akira Iguchi, Yoko Wada, Daisuke Kobayashi, Hiroe Sato, Tokuhide Oyama, Takeshi Nakatsue, Shuichi Murakami, Takeshi Kuroda, Masaaki Nakano & Ichiei Narita

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  1. Akira Iguchi
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  2. Yoko Wada
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  3. Daisuke Kobayashi
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  4. Hiroe Sato
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  5. Tokuhide Oyama
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  6. Takeshi Nakatsue
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  7. Shuichi Murakami
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  8. Takeshi Kuroda
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  9. Masaaki Nakano
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  10. Ichiei Narita
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Corresponding author

Correspondence to Akira Iguchi.

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Iguchi, A., Wada, Y., Kobayashi, D. et al. A case of MPO- and PR3-ANCA-positive hypertrophic cranial pachymeningitis with elevated serum IgG4. Mod Rheumatol 23, 151–155 (2013). https://doi.org/10.1007/s10165-012-0613-1

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  • DOI: https://doi.org/10.1007/s10165-012-0613-1

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