抄録
Wegener‘s granulomatosis (WG), more recently referred to as granulomatosis with polyangiitis (Wegener‘s) (GPA), is an incurable form of vasculitis that affects the nose, lungs, kidneys and other organs. Rhinitis is generally the first sign in most of the patients. Laryngeal involvement in GPA is often observed in subglottis. GPA of the larynx usually occurs concomitantly with the nasal presentation, and the PR3-ANCA (proteinase 3 antinuclear cytoplasmic antibodies) is typically positive. The positive rate of PR3-ANCA is however lower in patients with predominant granulomatosis manifestations limited to the upper respiratory tract. Unfortunately, many biopsies can be nonspecific and provide too little information for the diagnosis of GPA. If immunosuppressive treatment is not available, mortality within one year is over 80%. Administration of corticosteroids and cyclophosphamide dramatically improved prognosis of GPA. Survival rate of GPA in our hospital is in excess of 90%.
