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.
Highlights
IgG4-related disease is a newly designated disease entity, which can be defined as an idiopathic fibroinflammatory condition rich in IgG4+ plasma cells
A total of 21 peripheral nerve lesions were identified in 7 patients
Peripheral nerve lesions in ophthalmic area were always associated with IgG4-related dacryoadenitis or orbital disease, whereas spinal nerve lesions were isolated without IgG4-related disease in adjacent tissue
Summary
IgG4-related disease is a newly designated disease entity, which can be defined as an idiopathic fibroinflammatory condition rich in IgG4+ plasma cells. This disease affects a variety of organs including the salivary gland [1], pancreas [2], bile duct [3], lung [4], kidney [5], and aorta/artery [6, 7]. IgG4-related disease shares clinicopathological characteristics irrespective of the affected organs. IgG4-related disease predominantly develops in glandular organs, but nonglandular tissue like retroperitoneum can be affected as well [11, 12]
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