Internal Medicine
Online ISSN : 1349-7235
Print ISSN : 0918-2918
ISSN-L : 0918-2918
CASE REPORTS
Immunoglobulin G4-related Disease Accompanied by Peripheral Neuropathy: A Report of Two Cases
Tomoya KawazoeTomoyuki InoueShinsuke TobisawaKeizo SugayaToshio ShimizuKazuhito MiyamotoManaka GotoNaoto YokogawaSanami AzumaShingo ItagakiKenji NishidaYasuhiro MoritaMasahiro NagaoEiji Isozaki
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JOURNAL OPEN ACCESS

2021 Volume 60 Issue 12 Pages 1941-1947

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Abstract

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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© 2021 by The Japanese Society of Internal Medicine
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