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Intracranial immunoglobulin G4‐related disease successfully treated by steroid and oral cyclophosphamide: A case report
Author(s) -
Nishijima Haruo,
Arai Akira,
Kon Tomoya,
Funamizu Yukihisa,
Ueno Tatsuya,
Haga Rie,
Miki Yasuo,
Kimura Tamaki,
Suzuki Chieko,
Tomiyama Masahiko,
Kusumi Tomomi,
Minato Hiroshi,
Baba Masayuki
Publication year - 2013
Publication title -
neurology and clinical neuroscience
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.125
0ISSN - 2049-4173
DOI - 10.1002/ncn3.4
Subject(s) - medicine , cyclophosphamide , otitis , mastoidectomy , disease , dermatology , pathology , surgery , chemotherapy , cholesteatoma
Immunoglobulin G (IgG) 4‐related disease is a novel autoimmune disease that was discovered and established in the 21st century. We treated a patient, a 66‐year‐old Japanese woman, with exudative otitis media, pachymeningitis and orbital pseudotumor who showed poor responsiveness to glucocorticoid therapy, and was successfully treated by oral cyclophosphamide. We diagnosed her illness as intracranial IgG4‐related disease by immunohistochemical examination. Mastoidectomy was carried out 8 months after the first onset of exudative otitis media when the general concept of IgG4‐related disease was not established, and we re‐examined the specimen from her middle ear 5 years after the operation. This is the first report of a patient with histologically confirmed intracranial IgG4‐related disease who was successfully treated by cyclophosphamide. Oral cyclophosphamide can be effective to treat intracranial IgG4‐related disease that is resistant to steroid therapy.