Wegener's granulomatosis--a review of diagnosis and treatment in 53 subjects

Abstract

We reviewed 79 patients with clinically suspected Wegener's granulomatosis (WG) diagnosed in Nottingham between 1990 and 1997. Fifty-three patients were confirmed as having WG. We describe the symptoms and signs, features of diagnostic significance, ANCA results, biopsy sites, histology, treatment and outcome in this group. Nasal symptoms and signs had a positive predictive value of 63%, c-ANCA at presentation 100%, and positive nasal biopsy 100%. The negative predictive values were 41%; 79% and 74% respectively. From this study, we recommend that patients who have a negative ANCA and where there remains a clinical suspicion of WG, an ENT examination should be undertaken. Whilst a suspicious lesion should be biopsied and a positive histological picture has a 100% positive predictive value, a negative nasal biopsy does not exclude WG as 5 patients went on to develop a positive ANCA up to 4 years later. In 11 ANCA negative patients where there were signs of nasal mucosal disease, 6 had a positive biopsy and this highlights the importance of nasal biopsy. The c-ANCA should be repeated in patients with a negative c-ANCA and biopsy results at presentation, in whom there remains a clinical suspicion of WG.