AN UNUSUAL CLINICAL COURSE OF WEGENERS GR ANULOMATOSIS

Wegener's granulomatosis is an immunepathogenic disease of unknown ori gin. The histopathological picture shows granulomatous inflammation wi th epitheloid cells, granulomas, and general vasculitis. The diagnosis of Wegener's granulomatosis is made on the basis of the clinical pict ure, serum cANCA, and histologic examination of biopsies. Patient: We present the case of a 57-year-old white male patient who was admitted to our ENT Hospital with a six weeks' history of otalgia and incomplet e ipsilateral facial palsy since the day before admission. The patient had clinical features of acute otitis media without signs of mastoidi tis. Despite a ten days' course of intravenous antibiotic treatment, t he intensity of facial palsy progressed and the general condition of t he patient worsened. A mastoidectomy and decompression of the facial n erve were performed, demonstrating sclerosis of the mastoid cells. Thr ee weeks after release from the hospital, the patient was admitted aga in with recurrent fever, cephalea, loss of weight, and arthritic pain. There were no signs of recurrent otitis media or mastoiditis, and sig moid sinus thrombosis was ruled out. Even under aggressive, intravenou s antibiotic treatment the general physical condition continued to wor sen; septic temperatures and signs of beginning renal failure occurred . The patient was transferred to the ICU with the diagnosis of sepsis of unknown origin. There bloodtests were positive for cANCA, which is highly specific for Wegener's granulomatosis. Under therapy with cyclo phosphamide and IV corticosteroid, the patient recovered within 14 day s. Conclusion: The lack of symptoms in the upper respiratory tract in our patient was unusual, indicating that in patients with recurrent ot itis media, facial palsy, mastoiditis, or external otitis Wegener's gr anulomatosis should be ruled out as differential diagnosis.