Granulomatosis with polyangiitis (upper respiratory tract manifestations)

Dr Owen Kang and Dr Henry Knipe et al.

The upper respiratory tract manifestations of granulomatosis with polyangiitis (GPA) (previously known as Wegener granulomatosis) are common and affect most patients. . Granulomatosis with polyangiitis is a multisystem systemic necrotizing non-caseating granulomatous vasculitis affecting small to medium-sized arteries, capillaries and veins.

A rare disease predominantly affecting individuals in the 5th to 7th decades of life, with a slight predilection towards males.

The majority of patients of GPA have upper respiratory tract involvement, most commonly presenting with nasal obstruction, rhinitis and epistaxis 1,3 - akin to chronic rhinosinusitis. Often presents following a protracted period of symptoms (months to years), due to being mistaken for chronic sinusitis clinically.

GPA manifests in the upper respiratory tract with 1-3:

  • sinusitis/mastoiditis/otitis
  • sinonasal mucosal ulcers
  • bone deformities, e.g. nasal septal perforation, nasal saddle deformity
  • subglottic stenosis
  • non-contrast CT
    • sinonasal mucosal thickening +/- air-fluid levels +/- soft tissue nodules
    • bony/cartilaginous erosions, in particular, affecting lamina papyracea, nasal septum, and lateral nasal cavity wall +/- perforation
  • post-contrast CT
    • enhancing soft tissue nodules
  • T1: low-to-intermediate signal intensity nodules or mass-like mucosal lesions
  • T1 C+ (Gd): enhancement of involved mucosal tissue
  • T2
  • low signal intensity of nodules, relative to inflamed (oedematous) surrounding mucosa

Usually consists of a combination of corticosteroids and cytotoxic agents such as cyclophosphamide. Generally indolent, but if fulminant disease occurs with secondary sepsis, antibiotics are required.

Possible considerations include:

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Article information

rID: 43203
System: Head & Neck
Synonyms or Alternate Spellings:
  • Upper respiratory tract manifestations of Wegener granulomatosis
  • Upper respiratory tract manifestations of granulomatosis with polyangiitis (GPA)

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    Case 3: with sinus and nasal septal involvement
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