Granulomatosis with polyangiitis (upper respiratory tract manifestations)

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.

Epidemiology

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

Clinical presentation

The majority of patients of GPA have upper respiratory tract involvement, most commonly presenting with nasal obstruction, rhinitis and epistaxis - 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 :

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
  • 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.

Differential diagnosis

Possible considerations include:

Siehe auch: