Etiology • Unknown • A necrotizing vasculitis with preferential involvement of the
respiratory tract early in its course • Oral involvement unusual and characterized by ulceration and
tissue destruction
Clinical Presentation • Jaw pain, gingival inflammation with petechiae or hyperplasia,
palatal ulceration with possible perforation • Painful salivary gland enlargement may be encountered. • Classic triad of upper airway, lung, and kidney involvement
not required for diagnosis • May remain localized for prolonged periods prior to multi-
organ involvement
respiratory tract early in its course • Oral involvement unusual and characterized by ulceration and
tissue destruction
Clinical Presentation • Jaw pain, gingival inflammation with petechiae or hyperplasia,
palatal ulceration with possible perforation • Painful salivary gland enlargement may be encountered. • Classic triad of upper airway, lung, and kidney involvement
not required for diagnosis • May remain localized for prolonged periods prior to multi-
organ involvement
Microscopic Findings • Vasculitis, necrosis, and granulomatous inflammation
Diagnosis • Oral or upper airway biopsy is helpful in less than one-half of
cases. • Tissue biopsy for microscopic features • Laboratory studies show the following:
• Antineutrophil cytoplasmic antibodies (ANCAs)—two staining patterns: cytoplasmic ANCA (high specificity) and perinuclear ANCA
• Mild, normocytic, normochromic anemia (in 50%) • Elevated erythrocyte sedimentation rate
Differential Diagnosis • Lymphoma, including midline granuloma • Late-stage syphilis • Deep fungal infection • Tuberculosis • Major aphthous ulcers
Treatment • Cyclophosphamide/prednisone • Trimethoprim/sulfamethoxazole (as monotherapy or in
combination with immunosuppressive therapy)
Prognosis • Fair • Secondary complications related to long-term immuno-
suppression