[Wegener's granulomatosis involving head and neck: retrospective analysis of 16 patients]

Abstract

Based on a retrospective analysis of patients with Wegener's granulomatosis (WG), the aim of our study was to compare our finding with those previously reported in the literature. We also reviewed the characteristic features of WG, particularly otorhinolaryngology features. Sixteen consecutive patients were followed over a period of 23 years. There were 8 men and 8 women, all Caucasians, aged between 13 and 74 years (mean 42 years). Initial ENT signs and symptoms were observed in 62.5% of the cases, and occurred in 94% during disease progression with predominant rhino-sinusal (75%) and otological (38%) or laryngotracheal (25%) localizations. Later, the disease primarily affected the lower respiratory tract (87.5%) and the kidneys (50%). Articular (40%), ocular (25%), cardiovascular (25%), neurological (25%), and cutaneous (19%) localizations were also observed. The mean delay to diagnosis was 40 months, more than 1 year in 7 patients. A definitive diagnosis could not be made for 5 patients because of lack of histological evidence, but clinical and biological arguments suggested diagnosis of WG. Antineutrophil cytoplasmic antibodies (ANCA) were assessed in 13 patients and were positive in all. The role of ENT biopsies was significant to our study (31%). The ENT specialist can play a very important role in the diagnosis of Wegener's granulomatosis.