[Clinical significance of "ANCA" in the diagnosis of Wegener's granulomatosis: 8 years of experience]

Abstract

In its early stages Wegener's Granulomatosis (WG) commonly presents symptoms in E.N.T. sites. However, these symptoms are not specific and vary greatly so that alone they rarely lead to a correct diagnosis. Anti-neutrophil cytoplasmic autoantibodies (ANCA) have recently been found in patients with WG. The presence of such autoantibodies is now considered a specific test for this disorder. Over the last 8 years, 11 patients with WG having E.N.T. involvement were observed. All were tested for the presence of ANCA and such antibodies were found in 10 out of 11 patients. Biopsy showed the histopathological characteristics of WG--i.e. granulomatous necrotizing vasculitis--in five cases (in one patient biopsy was carried out surgically in the lung). Subglottic localization was surprisingly common in these patients, being seen nearly as frequently as the sinonasal localization (generally the most common manifestation of WG in the E.N.T. district). Early identification of the WG subglottic stenosis made it possible to administer appropriate medical therapy, thus limiting the number of surgical procedures. Only one patient was temporarily tracheostomized. None underwent laryngo-tracheoplasty. A major clinical problem in WG is to keep the interval between onset and definitive diagnosis as short as possible. At the outset, WG nearly always displays symptoms and signs in E.N.T. sites. Unfortunately, however, the diagnosis is nearly always made when pulmonary, or even worse renal, localizations appear. This considerably worsens the quoad vitam and quoad valetudinem prognosis. The authors' experience leads them to conclude that Anti-neutrophil cytoplasmic autoantibodies, in their C-ANCA pattern, represent a useful tool for prompt diagnosis of Wegener Granulomatosis, particularly in limited or atypical forms.