Head and Neck ImagingWegener's granulomatosis, head and neck manifestation
(Friedrich Wegener, 20th century, German pathologist), systemic disease primarily affecting the upper respiratory tract, lungs and kidneys. It is characterized by necrotizing granuloma, vasculitis and glomerulonephritis. Patients usually present with constitutional symptoms such as fever and weight loss. They may also have nasal discharge and sinusitis; clinical examination reveals a crusted, granular
mucosa, with
ulceration and bone destruction.
CT shows bone destruction in the nose and
paranasal sinuses, without a significant soft tissue mass (
Fig. 1). Similar, but more pronounced, abnormalities are seen in
lethal midline granuloma
.
In about 20% of cases, Wegener's granulomatosis also involves the orbit. Wegener's granulomatosis limited to the orbits is very rare. The orbital symptoms are the same as those of
orbital pseudotumour
but patients with Wegener's granulomatosis usually have constitutional symptoms. Wegener's granulomatosis may affect the larynx; soft tissue thickening causing subglottic or glottic
stenosis is seen in such cases (
Fig. 2). Soft tissue masses mimicking a
malignant tumour of the nasopharynx or skull base have also been described.
A positive titre for c-ANCA is a diagnostic criterion for Wegener's granulomatosis. This cytoplasmic antibody directed at neutrophil cytoplasmic antigen is elevated during periods of active disease. In the absence of respiratory tract or
renal lesions, the diagnosis cannot be confirmed until the elevated c-ANCA titers are identified. For a general description of the disease, see
Wegener's granulomatosis
.
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Axial CT image of nose. Septal perforations and defect in left inferior turbinate (arrowheads), without associated soft tissue thickening.
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Wegener's granulomatosis, head and neck manifestation, Fig. 1a | | Wegener's granulomatosis, head and neck manifestation, Fig. 2a | |