BMJ Case Reports 2017; doi:10.1136/bcr-2017-219897
  • Rare disease

IgG4-related disease: long-term natural history and management of a relapsing multisystem disease entity

  1. Philip Berry
  1. Department of Gastroenterology, Guy’s and Saint Thomas’ NHS Foundation Trust, London, UK
  1. Correspondence to Dr Philip Berry, philip.berry{at}
  • Accepted 19 May 2017
  • Published 16 July 2017


Although awareness of IgG4-related disease has grown over the past decade, with earlier diagnosis and treatment, understanding of its natural history over the long term and the optimal management remains unclear. We report the case of a 48-year-old man who presented with a pancreatic pseudotumour causing bile duct obstruction with coexisting autoimmune hepatitis and multisystem involvement. His symptoms settled on steroids and maintenance with azathioprine was commenced, however periodic relapses occurred involving multiple organs. A timeline-relating IgG4 levels, clinical features and immunosuppressive therapy are presented. The protean and relapsing–remitting nature of this condition is emphasised, and a brief review of long-term therapeutic options is provided.


  • Twitter @philaberry

  • Contributors All persons who meet authorship criteria are listed as authors, and all authors certify that they have participated sufficiently in the work to take public responsibility for the content, including participation in the concept, writing or revision of the manuscript. Furthermore, each author certifies that this material has not been submitted to or published in any other publication. PB: conception and revising the manuscript critically for important intellectual content. SK and PB: writing. All authors: revision; clinical management of the case and approval of the version of the manuscript to be published.

  • Competing interests None declared.

  • Patient consent Obtained.

  • Provenance and peer review Not commissioned; externally peer reviewed.

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