Immunoglobulin G4-related Disease Accompanied by Peripheral Neuropathy: A Report of Two Cases

Tomoya Kawazoe, Tomoyuki Inoue, Shinsuke Tobisawa, Keizo Sugaya, Toshio Shimizu, Kazuhito Miyamoto, Manaka Goto, Naoto Yokogawa, Sanami Azuma, Shingo Itagaki, Kenji Nishida, Yasuhiro Morita, Masahiro Nagao, Eiji Isozaki
Internal Medicine 2021 January 15
Due to its rarity and the limited literature, the clinicopathological characteristics of peripheral nerve involvement in immunoglobulin G4 (IgG4)-related disease are unknown. We present two cases of IgG4-related disease, accompanied by peripheral neuropathy, presenting as unilateral ptosis (case 1) and sclerosing cholangitis (case 2), respectively. In both cases, sural nerve biopsy indicated vasculitis as the underlying pathophysiology; the peripheral neuropathy was refractory to corticosteroid therapy. In contrast to the previously proposed pathomechanism of IgG4-related neuropathy (direct lymphoplasmacytic infiltration), the pathological findings in our cases suggest that vasculitis occurs secondary to systemic autoimmune conditions.

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