The objective of this prospective cross-sectional study is to describe the clinical otorhinolaryngological manifestations of granulomatosis with polyangiitis (Wegener's) (GPA) in a prospective cohort. All patients suffering from GPA seen in a tertiary centre between March 2007 and November 2008 had a detailed clinical assessment by an ENT surgeon of their ear, nose and head and neck complaints. An evaluation of whether there was disease activity and/or infection in each ENT area was made using the European Vasculitis Study Group guidelines. The number of patients assessed was 144. The proportion of female patients was 47 % (n = 69) and the median age was 57.7 years (IQ range 42.5-68.5). The prevalence of ENT involvement was 87 % (125/144). Hearing loss and abnormal tympanic membrane appearance were more common in patients with active disease and no infection (7/8 and 6/8, respectively, in active disease cf. 59/131 and 52/131, respectively, in remission). Nasal crusting was the most common nasal complaint recorded (52/144, 36 %) and bloody rhinorrhoea was the most common symptom in patients with disease activity. Rhinoscopy was highly sensitive in diagnosing disease activity (100 %). Subglottic stenosis was the most common head and neck manifestation (27/121, 22 %) and 74 % were symptomatic. In conclusion, the pattern and frequency of clinical ENT manifestations in GPA have been described in a large patient cohort. The use of tools readily available in the ENT clinic was essential to assess these patients accurately. This dataset will form the basis of an objective scoring system to measure disease activity in the ENT system.
European Archives of Oto-rhino-laryngology : Official Journal of the European Federation of Oto-rhino-laryngological Societies (eufos) : Affiliated With the German Society for Oto-rhino-laryngology - Head and Neck Surgery, 2013, Vol 270, Issue 1, p. 345-54