title: A case of MPO- and PR3-ANCA-positive hypertrophic cranial pachymeningitis with elevated serum IgG4.
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.
authors.affiliation: 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
authors.forename: Akira
authors.initials: A
authors.lastname: Iguchi
authors.forename: Yoko
authors.initials: Y
authors.lastname: Wada
authors.forename: Daisuke
authors.initials: D
authors.lastname: Kobayashi
authors.forename: Hiroe
authors.initials: H
authors.lastname: Sato
authors.forename: Tokuhide
authors.initials: T
authors.lastname: Oyama
authors.forename: Takeshi
authors.initials: T
authors.lastname: Nakatsue
authors.forename: Shuichi
authors.initials: S
authors.lastname: Murakami
authors.forename: Takeshi
authors.initials: T
authors.lastname: Kuroda
authors.forename: Masaaki
authors.initials: M
authors.lastname: Nakano
authors.forename: Ichiei
authors.initials: I
authors.lastname: Narita
mesh_terms: D019268:Antibodies, Antineutrophil Cytoplasmic / Q000097:blood* / Q000276:immunology; D001158:Arteries / Q000473:pathology; D001327:Autoimmune Diseases / Q000175:diagnosis; D003520:Cyclophosphamide / Q000627:therapeutic use; D003937:Diagnosis, Differential; D004351:Drug Resistance; D004359:Drug Therapy, Combination; D004388:Dura Mater / Q000473:pathology; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D006942:Hypergammaglobulinemia / Q000175:diagnosis; D007074:Immunoglobulin G / Q000276:immunology*; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008279:Magnetic Resonance Imaging; D008581:Meningitis / Q000097:blood / Q000175:diagnosis* / Q000188:drug therapy; D008875:Middle Aged; D009389:Neovascularization, Pathologic; D009918:Orbital Neoplasms / Q000098:blood supply / Q000150:complications / Q000473:pathology*; D009195:Peroxidase / Q000097:blood* / Q000276:immunology; D012421:Rupture; D016896:Treatment Outcome
chemical_list: D019268:Antibodies, Antineutrophil Cytoplasmic; D005938:Glucocorticoids; D007074:Immunoglobulin G; D007166:Immunosuppressive Agents; D003520:Cyclophosphamide; D009195:Peroxidase