It is now widely recognized that type 1 autoimmune pancreatitis (AIP), which pathologically corresponds to lymphoplasmacytic sclerosing pancreatitis (LPSP), is part of the IgG4-related disease (IgG4-RD) spectrum and is associated with a wide range of extrapancreatic lesions. However, little has been written to date about peripheral nerve involvement in this condition. We analyzed the relationship between type 1 AIP and infraorbital nerve swelling and identified a significantly higher frequency of nerve swelling as compared to that of a control group. In addition, we observed that the infraorbital nerve lesions responded well to glucocorticoid therapy. Infraorbital nerve swelling was also associated with other organ involvement typical of IgG4-RD, namely lacrimal, submandibular, and parotid gland disease and enlargement of the extraocular muscles. These results are consistent with the concept that infraorbital nerve swelling is a common peripheral nerve lesion in patients with type 1 AIP and IgG4-RD.
KeywordsLacrimal Gland Extraocular Muscle Nerve Lesion Autoimmune Pancreatitis Chronic Inflammatory Demyelinating Polyneuropathy
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