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Ear, nose and throat
ANCA-negative granulomatosis polyangiitis: an airway emergency
  1. Nita Rachel Rajan1,2,
  2. Nina Brown3,
  3. Edmond O'Riordan3 and
  4. Chaitanya Gadepalli1
  1. 1Department of ENT, Salford Care Organisation, Northern Care Alliance NHS foundation Trust, Salford, UK
  2. 2Department of ENT, Tameside and Glossop IC NHS Foundation trust, Ashton-under-Lyne, UK
  3. 3Department of Renal Medicine, Salford Care Organisation, Northern Care Alliance NHS foundation trust, Salford, UK
  1. Correspondence to Nita Rachel Rajan; nitanithin{at}gmail.com; Mr Chaitanya Gadepalli; chaitanya.gadepalli{at}nca.nhs.uk

Abstract

Granulomatosis polyangiitis (GPA) is an autoimmune condition causing inflammation of small blood vessels. It is a rare disorder that may affect various parts of the body. The diagnosis is often based on clinical examination, laboratory investigations and tissue biopsy. In about 10–20% of patients, the anti-neutrophilic cytoplasmic antibody (ANCA) can be negative, and histology maybe inconclusive, which can lead to diagnostic uncertainty. Failure to treat vasculitis can lead to morbidity and even mortality. We present a case report of a gentleman who was presented with an airway emergency with inflammation of the nasal cavity and subglottic involvement amounting to airway stenosis. His ANCA was negative and tissue biopsy from the subglottis was inconclusive. He underwent urgent dilatation of his airway, local therapies to the nose and was commenced on 10 cycles of cyclophosphamide. A follow-up of over 4 years has not shown any relapse of his disease clinically or biochemically. We discuss the clinical findings, diagnostic dilemma and multidisciplinary management of this life-threatening condition.

  • ear, nose and throat
  • immunological products and vaccines
  • renal system
  • vasculitis
  • healthcare improvement and patient safety

https://doi.org/10.1136/bcr-2023-258391

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    Footnotes

    • X @chai_gadepalli

    • Contributors NRR, CG,NB,ER were responsible for the drafting of the text, sourcing and editing of clinical images, investigation results, drawing original diagrams and algorithms, and critical revision for important intellectual content. CG and NRR are guarantors of this article.

    • 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.