title: Neurological Manifestations of IgG4-Related Disease.
abstract: 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.
authors.affiliation: Department of Internal Medicine, Hospital da Luz, Lisbon, Portugal.
authors.forename: Bernardo
authors.initials: B
authors.lastname: Baptista
authors.affiliation: Louise Coote Unit, Guy's and St Thomas NHS Foundation Trust, London, UK.
authors.forename: Alina
authors.initials: A
authors.lastname: Casian
authors.affiliation: Department of Rheumatology, Brunel Building, Southmead Hospital, Bristol, UK.|Musculoskeletal Research Unit, Learning and Research Building, University of Bristol, Southmead Hospital, Bristol, BS10 5NB, UK.
authors.forename: Harsha
authors.initials: H
authors.lastname: Gunawardena
authors.affiliation: Louise Coote Unit, Guy's and St Thomas NHS Foundation Trust, London, UK.|Division of Immunology, Infection and Inflammatory Diseases, King's College London, New Hunt's House, Guy's Campus, Great Maze Pond, London, SE1 1UL, UK.
authors.forename: David
authors.initials: D
authors.lastname: D'Cruz
authors.affiliation: School of Clinical Sciences, Level 1, Learning and Research Building, University of Bristol, Southmead Hospital, Bristol, BS10 5NB, UK. c.m.rice@bristol.ac.uk.|Department of Neurology, Brunel Building, Southmead Hospital, Bristol, UK. c.m.rice@bristol.ac.uk.
authors.forename: Claire M
authors.initials: CM
authors.lastname: Rice
keywords: Hypophysitis; IgG4-related disease; Neuropathy; Pachymeningitis; Pseudotumour