Wegener' s granulomatosis is a chronic idiopathic systemic disease. It is characterized by necrotizing granulomatous vasculitis that primarily involves the small arteries and veins. Involvement of the upper respiratory tract is the most common manifestation, and it is usually seen at the onset of the disease. Wegener's granulomatosis can also cause proliferative or focal necrotizing glomerulonephritis and disseminated angiitis, which can compromise any organ in the body.
Wegener' s granulomatosis can appear in patients of any age, but it typically manifests during middle adulthood (mean age of onset: 40 yr). It is rarely seen in blacks. It affects men and women equally, although a slight predilection toward men has been reported. (1) It is a potentially life-threatening disease, but complete remission can be achieved if it is diagnosed early and treated properly with immunosuppressants--cyclophosphamide and corticosteroids in particular. Otherwise, median survival is only 5 months. (1,2)
Ear, nose, and throat involvement in Wegener' s granulomatosis is the most common early clinical manifestation (92% of patients). The most common affected sites are the turbinates, nasal bones, and the nasal septum (figure). Among the signs and symptoms are persistent nasal obstruction, nasal drainage, sinus pain, epistaxis, hoarseness, otorrhea, and hearing loss. These conditions can be accompanied by systemic symptoms such as fever, malaise, night sweats, weight loss, and migratory arthralgias." (1,2) In severe and advanced cases, compromise of the orbital wall and other facial bones has been observed. (3)
On physical examination, findings can include a red dish and thickened septal mucosa, a septal perforation, and involvement of the nasal cartilage and/or bone, which can result in external deformities such as saddlenose. (2) The presence of hearing loss is related to recurrent serous or suppurative otitis media. (1) The laryngeal manifestation is subglottic involvement that results in stenosis. Subglottic involvement, which is seen in fewer than 20% of patients, is characterized by a reddish, friable, and circumferential narrowing in the subglottic area. Oral ulcers and bilateral parotid swelling might also be found." (1,2)
The diagnosis is made clinically and confirmed histopathologically. Predominant clinical findings are inflammation of the upper respiratory mucosa and submucosa, the presence of granulomas, and the presence of microabscesses accompanied by underlying vasculitis and extensive necrosis. (2)
The presence of mucosal thickening and inflammation of the nasal cavity and paranasal sinuses on computed tomography (CT) and magnetic resonance imaging (MRI) are early signs of Wegener's granulomatosis. In later stages, granuloma formation and ulceration can be seen. (3,4) Granuloma formations on MRI appear as low-signalintensity lesions on both T1- and T2-weighted images. (4) On CT, both bone destruction and new bone formation can be seen. CT, with its high degree of spatial resolution for bone, and MRI, with its high degree of contrast resolution for soft tissue, are complementary in imaging patients with Wegener's granulomatosis.
References
(1.) Hoffman GS, Kerr GS, Leavitt RY, et al. Wegener granulomatosis: An analysis of 158 patients. Ann Intern Med 1992;116:488-98.
(2.) Vartiainen E, Nuutinen J. Head and neck manifestations of Wegener's granulomatosis. Ear Nose Throat J 1992~71:423-4, 427-8.
(3.) Lloyd G, Lund VJ, Beale T, Howard D. Rhinologic changes in Wegener's granulomatosis. J Laryngol Otol 2002;116:565-9.
(4.) Muhle C, Reinhold-Keller E, Richter C, et al. MRI of the nasal cavity, the paranasal sinuses and orbits in Wegener's granulomatosis. Eur Radiol 1997;7:566-70.
From the Department of Radiology, Louisiana State University Health Science Center, New Orleans.
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