INTRODUCTION - Wegener's granulomatosis is a disease of unknown origin classified as an immune vasculitis. The main pathologic feature is necrotising granulomatous small vessel vasculitis. Clinically the upper and/or lower airways are affected most often as well as the kidneys in the generalised form. The highly specific antineutrophil cytoplasmic antibodies have diagnostic significance beside the pathology and clinical symptoms. PATIENTS AND METHODS - We have studied the clinical and diagnostic characteristics of 15 cases diagnosed in our centre from the last 25 years. All patients had pulmonary manifestation. We analysed the organ involvement, clinical signs indicating the diagnosis, chest radiography, chances for biopsies of diagnostic significance and the difficulties of differential diagnostics. RESULTS - The diagnosis was made based on histological samples from the upper airways in three cases and from the lower airways in six cases (in four cases the bronchoscopic biopsies were diagnostic, in two patients thoracotomy was necessary). In six cases, clinical signs and typical organ manifestations were the basis of the diagnosis. CONCLUSION - This very rarely occurring disease which is difficult to recognise requires pulmonological examination due to the involvement of lungs. If this diagnosis comes to mind - based on findings of bilateral, multiplex, round pulmonal shadows with cavitation - the pulmonologist should search for other organ manifestations and involve respective specialists. Ideally, diagnosis is established by histopathology or by positive cytoplasmic antineutrophil cytoplasmic antibodies.
|Number of pages||7|
|Journal||Lege Artis Medicinae|
|Publication status||Published - Oct 30 2001|
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