IgG4-related perineural disease

Dai Inoue, Yoh Zen, Yasuharu Sato, Hitoshi Abo, Hiroshi Demachi, Akio Uchiyama, Toshifumi Gabata, Osamu Matsui

Research output: Contribution to journalArticle

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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 30mm 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.

Original languageEnglish
Article number401890
JournalInternational Journal of Rheumatology
Volume2012
DOIs
Publication statusPublished - 2012

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Immunoglobulin G
Peripheral Nerves
Peripheral Nervous System Diseases
Optic Nerve
Neurologic Manifestations
Plasma Cells
Prefrontal Cortex
Steroids

ASJC Scopus subject areas

  • Rheumatology
  • Immunology

Cite this

Inoue, D., Zen, Y., Sato, Y., Abo, H., Demachi, H., Uchiyama, A., ... Matsui, O. (2012). IgG4-related perineural disease. International Journal of Rheumatology, 2012, [401890]. https://doi.org/10.1155/2012/401890

IgG4-related perineural disease. / Inoue, Dai; Zen, Yoh; Sato, Yasuharu; Abo, Hitoshi; Demachi, Hiroshi; Uchiyama, Akio; Gabata, Toshifumi; Matsui, Osamu.

In: International Journal of Rheumatology, Vol. 2012, 401890, 2012.

Research output: Contribution to journalArticle

Inoue, D, Zen, Y, Sato, Y, Abo, H, Demachi, H, Uchiyama, A, Gabata, T & Matsui, O 2012, 'IgG4-related perineural disease', International Journal of Rheumatology, vol. 2012, 401890. https://doi.org/10.1155/2012/401890
Inoue D, Zen Y, Sato Y, Abo H, Demachi H, Uchiyama A et al. IgG4-related perineural disease. International Journal of Rheumatology. 2012;2012. 401890. https://doi.org/10.1155/2012/401890
Inoue, Dai ; Zen, Yoh ; Sato, Yasuharu ; Abo, Hitoshi ; Demachi, Hiroshi ; Uchiyama, Akio ; Gabata, Toshifumi ; Matsui, Osamu. / IgG4-related perineural disease. In: International Journal of Rheumatology. 2012 ; Vol. 2012.
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