Wegener's granulomatosis. Long-term results of treatment

Seventeen patients with Wegener's granulomatosis are presented. The very complex clinical features are outlined. A recently suggested new classification (ELK-classification) is applied. Fourteen patients were treated with cytotoxic agents and steroids, 12 of these receiving 6-mercaptopurine. Of the ten patients still alive, eight are in remission with treatment withdrawn in three cases. The duration of the treatment is discussed. The results are largely satisfactory, but the course of the disease is still capricious. Progression to a higher step in the ELK-classification has been observed in several cases and a number of serious sequelae to the disease are recorded. The initial symptoms of Wegener's granulomatosis are varied and uncharacteristic, and it is important to bear this disease in mind when patients with a long course of apparently trivial infections or peculiar constellations of symptoms from several organs are encountered. Repeated biopsies from the respiratory tract are important in order to establish the diagnosis, but treatment should not be delayed in cases where only a tentative diagnosis can be made on the basis of a reasonably typical clinical picture, even with a negative histological response.