Granulomatosis with polyangiitis (upper respiratory tract manifestations)

Last revised by Yuranga Weerakkody on 10 May 2022

The upper respiratory tract manifestations of granulomatosis with polyangiitis (GPA) are common and affect most patients. 

For a general discussion of the condition, please refer to the main article on granulomatosis with polyangiitis (GPA). For other organ-specific radiographic features, please refer to individual articles:

Granulomatosis with polyangiitis (previously known as Wegener granulomatosis), is a multi-system 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:

  • 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
    • scalloping, sclerosis, neo-osteogenesis, and calcification may also be present.
  • 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 (edematous) surrounding mucosa

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

Possible considerations include:

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