[Wegener's granulomatosis and Wegener's Granuloma (author's transl)]

Abstract

The classical type of Wegener's granulomatosis encompasses a necrotizing granulomatous vasculitis of the upper and lower respiratory tracts and a necrotizing glomerulonephritis. As a rule, an initial involvement of the upper respiratory system in the nasal mucosa and paranasal sinuses is found. The terms "granuloma gangraenescens" and "midline disease" are frequently used at this stage of the disease. However, they should be abandoned and "Wegener's granuloma" preferred instead. A limited form of Wegener's granulomatosis has been described with pulmonary involvement which progresses without renal involvement. However, confinement of the disease to the upper respiratory system remains a controversial matter.