• Akira Iguchi, Yoko Wada, Daisuke Kobayashi, Hiroe Sato, Tokuhide Oyama, Takeshi Nakatsue, Shuichi Murakami, Takeshi Kuroda, Masaaki Nakano, and Ichiei Narita.
• 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. igucci4444@yahoo.co.jp
• Mod Rheumatol. 2013 Jan 1; 23 (1): 151-5.

AbstractWe 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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