Granulomatose mit Polyangiitis Manifestationen oberer Respirationstrakt

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: pulmonary manifestations
• granulomatosis with polyangiitis: renal manifestations
• granulomatosis with polyangiitis: CNS manifestations
• granulomatosis with polyangiitis: orbital manifestations

​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 :

• sinusitis/mastoiditis/otitis
• sclerosing osteitis of the nasal cavity
• sinonasal mucosal ulcers
• 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
• 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:

• sinusitis
  • chronic sinusitis
  • fungal sinusitis
• sinonasal polyposis
• sinonasal sarcoidosis
• nasal necrosis due to cocaine
• other causes of nasal septal perforation

Siehe auch:

• Granulomatose mit Polyangiitis