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Case Reports: Unusual association of diseases/symptoms
A unique presentation of IgG4 disease with ocular, neurologic and mastoid involvement
  1. Heena Narotam Jeena1,2,
  2. Amir Afrogheh3,4,
  3. Joshua West5,6,
  4. Freddie van der Colff7,8 and
  5. Naeem Brey1,2
  1. 1Division of Neurology, Stellenbosch University Faculty of Medicine and Health Sciences, Cape Town, Western Cape, South Africa
  2. 2Neurology, Tygerberg Hospital, Cape Town, Western Cape, South Africa
  3. 3Anatomical Pathology, Stellenbosch University Faculty of Medicine and Health Sciences, Cape Town, Western Cape, South Africa
  4. 4NHLS, Tygerberg Hospital, Cape Town, Western Cape, South Africa
  5. 5Otorhinolaryngology, Tygerberg Hospital, Cape Town, Western Cape, South Africa
  6. 6Otorhinolaryngology, Stellenbosch University Faculty of Medicine and Health Sciences, Cape Town, Western Cape, South Africa
  7. 7Ophthalmology, Stellenbosch University Faculty of Medicine and Health Sciences, Cape Town, Western Cape, South Africa
  8. 8Ophthalmology, Tygerberg Hospital, Cape Town, Western Cape, South Africa
  1. Correspondence to Dr Heena Narotam Jeena; heena.narotam.hn{at}gmail.com

Abstract

A man in his 20s presented with headache and acute deterioration in visual acuity. He was found to have panuveitis and raised intracranial pressure with papilloedema. MRI and F-fluorodeoxyglucose positron emission tomography confirmed a subclinical, but active, inflammatory mastoid process. Histology of the mastoid showed immunoglobulin G4 (IgG4) cells, plasma cells and storiform fibrosis.

This presentation of IgG4 disease has not been previously described.

Treatment with high-dose steroids was initiated, followed by long-term immunosuppressive therapy. The patient’s symptoms improved, although he remains dependent on azathioprine and low dose oral steroids for symptom control. To date, there has been no progression of the disease.

  • Ear, nose and throat/otolaryngology
  • Neurology
  • Ophthalmology
  • Pathology

https://doi.org/10.1136/bcr-2022-251736

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    Footnotes

    • Contributors HNJ: original layout of the study and composition of the manuscript. NB: critical revision of manuscript. JW: provided a perspective on the clinical and intra-operative findings. AA: head and neck pathologist who provided a perspective on the histology findings. FvdC: initial ophthalmological assessment and management.

    • 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.