title: IgG4-Related Perineural Disease.
abstract: Aims. To elucidate characteristics of IgG4-related disease involving the peripheral nervous system. Methods. Retrospective review of 106 patients with IgG4-related disease identified 21 peripheral nerve lesions in 7 patients. Clinicopathological and radiological features were examined. Results. Peripheral nerve lesions were commonly identified in orbital or paravertebral area, involving orbital (n = 9), optic (n = 4), spinal (n = 7), and great auricular nerves (n = 1). The predominant radiological feature was a distinct perineural soft tissue mass, ranging 8 to 30 mm in diameter. Histologically, the epineurium was preferentially involved by massive lymphoplasmacytic infiltration rich in IgG4(+) plasma cells. All lesions were neurologically asymptomatic and steroid-responsive at the first presentation, but one recurrent lesion around the optic nerve caused failing vision. Conclusion. IgG4-related disease of the peripheral nervous system is characterized by orbital or paravertebral localization, perineural mass formation, and rare neurologic symptoms. The term "IgG4-related perineural disease" seems appropriate to describe this entity.
authors.affiliation: Department of Radiology, Kanazawa University Graduate School of Medical Science, Kanazawa 920-8641, Japan.
authors.forename: Dai
authors.initials: D
authors.lastname: Inoue
authors.forename: Yoh
authors.initials: Y
authors.lastname: Zen
authors.forename: Yasuharu
authors.initials: Y
authors.lastname: Sato
authors.forename: Hitoshi
authors.initials: H
authors.lastname: Abo
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Demachi
authors.forename: Akio
authors.initials: A
authors.lastname: Uchiyama
authors.forename: Toshifumi
authors.initials: T
authors.lastname: Gabata
authors.forename: Osamu
authors.initials: O
authors.lastname: Matsui