Wegener's granulomatosis. The great masquerade: a clinical presentation and literature review.

Wegener's granulomatosis is classically a systemic disease which has masqueraded as many other disorders. The patient presenting with head and neck manifestations is often initially referred to the otolaryngologist. Although the natural history of this disease is fairly well understood, its etiology and pathogenesis are not clear. The response of Wegener's granulomatosis to appropriate therapy can be dramatic, whereas without treatment it is a progressive and mutilating disorder with life-threatening complications. Two cases which were recently managed at our center are presented in an attempt to illustrate the complexity of this disorder and the diagnostic and therapeutic dilemma which it presents. A literature review was carried out and a recent concept, the Multistep Evolution Hypothesis, as well as diagnostic advances in serological testing, are discussed. Cyclophosphamide and systemic steroids, the mainstay of therapeutic intervention, as well as more recent modalities including plasmapheresis, folate antagonists, oral trimethoprim-sulfamethoxazole and local radiotherapy are reviewed.