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An In Vivo Murine Sciatic Nerve Model of Perineural Invasion
Published on: April 23, 2018
IgG4-Related Perineural Disease.
Dai Inoue1, Yoh Zen, Yasuharu Sato
1Department of Radiology, Kanazawa University Graduate School of Medical Science, Kanazawa 920-8641, Japan.
International Journal of Rheumatology
|April 24, 2012
Summary
Immunoglobulin G4-related disease (IgG4-RD) can affect the peripheral nervous system, presenting as perineural masses. While often asymptomatic, these lesions can cause vision loss if they recur.
Area of Science:
- Neurology
- Immunology
- Pathology
Background:
- Immunoglobulin G4-related disease (IgG4-RD) is a multisystem fibroinflammatory condition.
- Peripheral nervous system involvement in IgG4-RD is uncommon but recognized.
Purpose of the Study:
- To characterize the clinicopathological and radiological features of IgG4-related disease affecting the peripheral nervous system.
Main Methods:
- Retrospective review of 106 patients with IgG4-RD.
- Analysis of 21 peripheral nerve lesions in 7 patients.
- Examination of clinicopathological and radiological data.
Main Results:
- Lesions commonly occurred in orbital or paravertebral areas, involving cranial and spinal nerves.
- Radiologically, a distinct perineural soft tissue mass (8-30 mm) was predominant.
- Histology showed epineural lymphoplasmacytic infiltration with IgG4(+) plasma cells.
- Most lesions were asymptomatic and steroid-responsive; one optic nerve lesion recurred, causing vision loss.
Conclusions:
- IgG4-related disease of the peripheral nervous system typically presents with orbital or paravertebral localization and perineural mass formation.
- Neurologic symptoms are rare, but recurrence can lead to significant deficits.
- The term "IgG4-related perineural disease" is proposed to describe this entity.
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