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IgG4-related disease of the mastoid: a rare presentation of a novel diagnosis
  1. Mohit Achanta1,
  2. Hisham Jaber2,
  3. Adaku Onovo3 and
  4. Ben Stew4
  1. 1Department of Otolaryngology, Imperial College Healthcare NHS Trust, London, UK
  2. 2Department of Radiology, University of Wales Hospital, Cardiff, UK
  3. 3Department of Cellular Pathology, University of Wales Hospital, Cardiff, UK
  4. 4Department of Otolaryngology, University of Wales Hospital, Cardiff, UK
  1. Correspondence to Dr Mohit Achanta; mohit.achanta{at}


IgG4-related disease (IgG4-RD) is an inflammatory condition characterised by infiltration of tissue by IgG4-positive plasma cells. This is the seventh reported case of IgG4-RD affecting the mastoid and informs clinicians in diagnosing patients affected by this rare condition.

A woman in her 20s presented with unilateral otalgia, hearing loss and vertigo. She deteriorated despite antibiotic therapy and cross-sectional imaging revealed a destructive extra-axial lesion of the mastoid cells. Biopsy confirmed a diagnosis of IgG4-RD. She was successfully treated with prednisolone and azathioprine.

Inflammatory conditions should be considered in patients with persistent middle ear symptoms after infection and malignancy are excluded. Delays in diagnosis can lead to irreversible mass effects and may occur as current diagnostic criteria exclude mastoid-specific features.

IgG4-RD remains a rare diagnosis. To avoid significant effects on a patients’ quality of life, prompt multidisciplinary treatment is vital alongside development of diagnostic criteria specific to otolaryngology.

  • Immunology
  • Head and neck surgery
  • Otolaryngology / ENT
  • Radiology
  • Rheumatology

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  • Contributors The following authors were responsible for drafting of the text, sourcing and editing of clinical images, investigation results, drawing original diagrams and algorithms, and critical revision for important intellectual content: MA, HJ, AO and BS. The following authors gave final approval of the manuscript: BS.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Case reports provide a valuable learning resource for the scientific community and can indicate areas of interest for future research. They should not be used in isolation to guide treatment choices or public health policy.

  • Competing interests None declared.

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