Granulomatosis with polyangiitis (upper respiratory tract manifestations)

Last revised by Yuranga Weerakkody on 10 May 2022

Citation, DOI, disclosures and article data

Citation:

Knipe H, Weerakkody Y, Bell D, et al. Granulomatosis with polyangiitis (upper respiratory tract manifestations). Reference article, Radiopaedia.org (Accessed on 18 Apr 2024) https://doi.org/10.53347/rID-43203

DOI:

https://doi.org/10.53347/rID-43203

Permalink:

https://radiopaedia.org/articles/43203

rID:

43203

Article created:

28 Feb 2016, Henry Knipe ◉ ◈

Disclosures:

At the time the article was created Henry Knipe had no recorded disclosures.

View Henry Knipe's current disclosures

Last revised:

10 May 2022, Yuranga Weerakkody ◉

Disclosures:

At the time the article was last revised Yuranga Weerakkody had no financial relationships to ineligible companies to disclose.

View Yuranga Weerakkody's current disclosures

Revisions:

22 times, by 14 contributors - see full revision history and disclosures

Systems:

Head & Neck

Tags:

cases, snippet title

Synonyms:

Upper respiratory tract manifestations of Wegener granulomatosis
Upper respiratory tract manifestations of granulomatosis with polyangiitis (GPA)

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.

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.

Pathology

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

sinusitis/mastoiditis/otitis
sclerosing osteitis of the nasal cavity ⁴
sinonasal mucosal ulcers
lacrimal gland involvement
bone deformities, e.g. nasal septal perforation, nasal saddle deformity
subglottic stenosis

Radiographic features

CT

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

MRI

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

Treatment and prognosis

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.