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International Journal of Rheumatology
Volume 2012 (2012), Article ID 401890, 9 pages
Clinical Study

IgG4-Related Perineural Disease

1Department of Radiology, Kanazawa University Graduate School of Medical Science, Kanazawa 920-8641, Japan
2Department of Radiology, Toyama Prefectural Central Hospital, Toyama 930-8550, Japan
3Institute of Liver Studies, King’s College Hospital, London 5E5 9RS, UK
4Department of Pathology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama 700-8558, Japan
5Department of Pathology, Toyama Prefectural Central Hospital, Toyama 930-8550, Japan

Received 24 November 2011; Accepted 13 January 2012

Academic Editor: John H. Stone

Copyright © 2012 Dai Inoue et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.


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.