Immunoglobulin G4-related disease presenting with peripheral neuropathy: a case report

Abstract

Background: Immunoglobulin G4-related disease (IgG4-RD) is an immune-mediated fibro-inflammatory condition characterized by high serum IgG4 concentrations and tissue infiltration by IgG4-positive plasma cells. Reports have demonstrated that IgG4-RD affects various organs, including the pancreas, kidney, lung, thyroid, and lacrimal and salivary glands. In the nervous system, hypertrophic pachymeningitis and hypophysitis are mainly related to IgG4-RD; however, the peripheral neuropathy involvement is unusual. Case presentation: We report on a 69-year-old woman with multiple mononeuropathy, weight loss and kidney mass in the setting of IgG4-RD. Biopsies of the kidney mass showed lymphoplasmacytic sclerosing inflammation with numerous IgG4-positive plasma cells. IgG4 and IgG4/IgG ratios in the blood were elevated. The patient was treated with high dose methylprednisolone with improvement in her neuropathy. Conclusions: IgG4-RD is a relatively recently reported systemic fibrous inflammatory disease caused by the infiltration of IgG4-positive plasma cells in various organs. In the nervous system, symptomatic peripheral nerve invasion is very rare. However, as demonstrated in our case, IgG4-RD may present with primarily peripheral nerve disease.