Ear, nose, and throat symptoms in subacute Wegener's granulomatosis

The standard description of Wegener's granulomatosis emphasises renal failure and thus a distorted impression may be given. Subacute and even chronic cases occur, and in these patients the presentation is varied and often insidious, leading to delay in diagnosis. Twenty two such patients (13 women and nine men) with a mean age of 44 years were seen in our connective tissue disease clinic. The mean duration of symptoms before diagnosis was 3.6 years and the mean duration of disease 5.9 years (19 years in one patient). All patients had malaise and ear, nose, and throat symptoms, and most had joint pains. Impaired renal function was seen in seven patients only. Tissue biopsy was diagnostic in half of the patients, and appreciably high titres of antineutrophil cytoplasmic antibodies were detected in only nine of 18 patients in whom these were measured. The most useful investigations were neutrophil counts, chest radiographs, and computed tomography of the sinuses and orbits. The most effective treatment was with intravenous pulses of cyclophosphamide. No deaths occurred. At the time of writing two patients were in remission and no longer being treated and 18 patients were in partial remission on continued treatment. Patients with subacute forms of Wegener's granulomatosis present with a variety of clinical features and the insidious presentation often leads to delay in diagnosis. A history of ear, nose, and throat symptoms was universal in our patients.