Artikel
Fulminant Wegener's granulomatosis course with introductory ENT symptoms (case report)
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Veröffentlicht: | 19. April 2011 |
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Wegener's granulomatosis is autoimmune systemic disease of unknown aetiology characterized by disseminated necrotizing vasculitis of minor and intermediate vessels in respiratory tract, kidneys and other organs. If untreated the mortality is over 90%. Localized ENT symptoms in nose are: nasal obstruction, mucosa hypertrophy, serous secretion, bleeding, septum perforation, chronic rhinosinusitis, in ear acute or chronic catarrhal otitis media, perceptive, conductive or mixed hypacusis. Among non-ENT symptoms belong cough, haemoptysis, dyspnoea, respiratory failure in lungs, haematuria and nephrotic syndrome in kidney, hypertrophic pachymeningitis, paresis of cranial nerves (II, VI, VII, VIII) in CNS. Also the general non-specific symptoms as fatigue, weakness, muscles and joints pain, night sweating, weight loss etc. are often described. We present a case report of man aged 29 admitted to the ENT department with prolonged otitis media on the right with mild paresis of facial nerve. Antibiotics and corticoids were applied. HRCT displayed inflammatory changes in right temporal bone without osteolytic signs, MRI showed fluid collection and possible abscess in middle temporal fossa. Antromastoidectomy was performed with conclusion of mastoiditis with reactive facial nerve paresis and circumscript pachymeningitis. After therapy the patient recovered and was discharged. Four days after discharge the fever over 38°C, CRP elevation, nasal obstruction, incomplete paresis of nerve IX contralaterally occurred and subsequently also cough with blood expectoration, dyspnoea and septic fever followed. HRCT showed extensive lung changes, cANCA antibodies were strongly positive. Despite treatment the patient died of acute respiratory failure 11 days after recurrence of the illness.