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Case Reports
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, and Eiji Isozaki.
- Department of Neurology, Tokyo Metropolitan Neurological Hospital, Japan.
- Intern. Med. 2021 Jun 15; 60 (12): 1941-1947.
AbstractDue 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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