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Case report
Treatment-resistant cough: a rare manifestation of IgG4-related disease involving the larynx
  1. Azfar Shahid Syed1,
  2. Rhonda E Colombo2,
  3. Bakhtiar S Syed3 and
  4. Peter M Henning4
  1. 1Internal Medicine, Madigan Army Medical Center, JBLM, Tacoma, Washington, USA
  2. 2Internal Medicine/Infectious Disease, Madigan Army Medical Center, JBLM, Tacoma, Washington, USA
  3. 3Gastroenterology, Yale New Haven Health System, New Haven, Connecticut, USA
  4. 4Internal Medicine/Rheumatology, Madigan Army Medical Center, JBLM, Tacoma, Washington, USA
  1. Correspondence to Dr Azfar Shahid Syed; azfar.shahid.syed{at}gmail.com

Abstract

IgG4-related autoimmune diseases (IgG4 RD) are a relatively recently recognised group of disease processes that can affect multiple organ systems and result in protean symptoms. Here, we present a rare case of a 69-year-old man with a history of IgG4 RD affecting his lacrimal gland and pancreas who developed symptoms of severe laryngitis not responsive to usual therapy. He presented with non-productive cough, hoarseness and dyspnoea. Imaging findings suggestive of aortitis and laryngeal inflammation in the setting of his IgG4 RD history prompted treatment with rituximab, which resulted in resolution of his laryngeal symptoms. Subsequently, his cough returned and he required periodic rituximab infusions to stay symptom-free. IgG4 RD of the larynx is an uncommonly reported manifestation in literature. This disease is very responsive to anti-CD20 monoclonal antibody treatment. IgG4 RD should be considered in patients with airway symptoms that are especially refractory to usual therapy.

  • rheumatology
  • ear
  • nose and throat

https://doi.org/10.1136/bcr-2020-237614

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    Footnotes

    • Presented at This case was previously presented as a poster at the American College of Physicians (ACP) national conference in Philadelphia in 2019 and at the ACP Washington State regional conference in 2018.

    • Contributors ASS wrote the majority of the case and contributed to writing the introduction and discussion, along with the final editing of the manuscript. RC assisted with writing the introduction along with editing the final manuscript. BSS assisted in writing the discussion and assisted with editing the final manuscript. PH was the physician taking care of the patient. He assisted with writing the abstract along with editing the manuscript.

    • 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.

    • Disclaimer The views expressed are those of the author(s) and do not reflect the official policy or position of the US Army Medical Department, Department of the Army, Department of Defense or the US Government.

    • Competing interests None declared.

    • Patient consent for publication Obtained.

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