IgG4-Related Perineural Disease

Abstract EN

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.