Neurological Manifestations of IgG4-Related Disease

Bernardo Baptista, Alina Casian, Harsha Gunawardena, David D'Cruz, Claire M Rice

Research output: Contribution to journalArticle (Academic Journal)peer-review

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IgG4-related disease (IgG4-RD) is a multisystem inflammatory disorder. Early recognition of IgG4-RD is important to avoid permanent organ dysfunction and disability. Neurological involvement by IgG4-RD is relatively uncommon, but well recognised—hypertrophic pachymeningitis and hypophysitis are the most frequent manifestations. Although the nervous system may be involved in isolation, this more frequently occurs in conjunction with involvement of other systems. Elevated circulating levels of IgG4 are suggestive of the condition, but these are not pathognomonic and exclusion of other inflammatory disorders including vasculitis is required. Wherever possible, a tissue diagnosis should be established. The characteristic histopathological changes include a lymphoplasmacytoid infiltrate, storiform fibrosis and obliterative phlebitis. IgG4-RD typically responds well to treatment with glucocorticoids, although relapse is relatively common and treatment with a steroid-sparing agent or rituximab may be required. Improved understanding of the pathogenesis of IgG4-RD is likely to lead to the development of more specific disease treatments in the future.
Original languageEnglish
Pages (from-to)14
Number of pages25
JournalCurrent Treatment Options in Neurology
Issue number4
Early online date3 Apr 2017
Publication statusPublished - 30 Apr 2017

Bibliographical note

Special Issue: Topical Collection on Neurologic Manifestations of Systemic Disease


  • IgG4-related disease
  • Pachymeningitis
  • Hypophysitis
  • Pseudotumour
  • Neuropathy


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