Vol.23 No.1

Case Report

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

Authors

Akira Iguchi1,2,3,4 , Yoko Wada1,2,3,4 , Daisuke Kobayashi1,2,3,4 , Hiroe Sato1,2,3,4 , Tokuhide Oyama1,2,3,4 , Takeshi Nakatsue1,2,3,4 , Shuichi Murakami1,2,3,4 , Takeshi Kuroda1,2,3,4 , Masaaki Nakano1,2,3,4 , Ichiei Narita1,2,3,4

  • 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
  • Division of Ophthalmology and Visual Sciences, Niigata University Graduate School of Medical and Dental Sciences, Niigata, Japan
  • Health Administration Center of Niigata University, Niigata, Japan
  • Department of Medical Technology, Faculty of Medicine, School of Health Sciences, Niigata University, Niigata, Japan
Received:

3 October 2011

Accepted:

2 February 2012

Published online:

15 February 2012

Full Text

PDF　(member's only)

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.

Key words

ANCA - Cyclophosphamide - IgG4 - Orbital tumor - Pachymeningitis