Nasal manifestations of wegener's granulomatosis

Abstract

A review of 52 recent cases of Wegener's granulomatosis has clarified the clinical manifestations and has emphasized the otolaryngologist's role in its early diagnosis. All the cases satisfied the currently accepted criteria for the condition: the presence of primary necrotizing vasculitis in a patient with a typical clinical course. Among the 52 patients, 31 patients had nasal lesions. The typical manifestation was persistent nasal obstruction in a middle‐aged patient with no history of nasal disorder. In the early stages, intranasal examination may reveal only diffuse nasal mucosal swelling, without tissue destruction. Systemic symptoms are malaise, night sweats, intermittent pyrexia, and migratory arthralgias; an abnormally high sedimentation rate is also frequent. Later, the typical nasal findings are diffuse destruction of the mucosa and foul‐smelling crusts. The tissue underlying the crusts is extremely friable. Perforations of the nasal septum are also common. Adequate biopsy of representative tissue is important. The condition is treatable; currently, corticosteroids with or without cyclophosphamide are being used. Of the 52 patients, 31 are alive; 10 are dead, and current information is unavailable on 11 patients.Polymorphic reticulosis (PMR, lethal midline granuloma, or malignant midline reticulosis) was noted in 16 other patients during the same period. Differentiation between PMR and Wegener's granulomatosis is important because, although they can resemble each other clinically, they are morphologically distinct and respond to different forms of therapy (Wegener's granulomatosis to immunosuppressive drugs and PMR to irradiation).The otolaryngologist has an important role in the early diagnosis of Wegener's granulomatosis because of the high incidence of nasal involvement and because of the variations in clinical manifestations, which depend on the stage of the disease.