Original paper
Wegener’s granulomatosis – observation of cases

Wegener’s granulomatosis (WG) belongs to the group of necrotising granulomatous vasculitides. It affects primarily small and medium size arteries, although the involvement of capillaries and veins is frequent as well. Epidemiological data show increased incidence of Wegener’s granulomatosis, particularly in northern Europe during recent years. Early diagnosis of WG is difficult. We present a retrospective analysis of 7 patients diagnosed with WG and treated at the Central Clinical Hospital in Warsaw in the years 1998–2006. The period of observation was 1–9 years. Changes resolved quickly under standard treatment. In all patients general symptoms of fever, weight loss and progressive weakness were present. In 4 persons the disease began with arthralgias or arthritis, in 4 with cough or haemoptysis, and in 1 with ocular signs. In our patients upper airway involvement was present in 5 cases (75%) and lower airway involvement in 5 (75%). All WG cases observed by us were of severe course with renal involvement. Apart from standard treatment in 1 patient with rapidly progressive renal failure dialyses were applied for 2.5 years and kidney transplantation was performed in the 3rd year from diagnosis. Two patients died in the course of observation: 1 from cardiac arrest due to terminal renal failure and 1 from subarachnoid haemorrhage penetrating to brain chambers (III and IV and occipital horns of both lateral chambers). The duration of remission was between 1 and 9 years. Relapses occurred in 3 patients. The prevalence of WG in proportion to all administrations to our hospital, which is a full-scale clinical unit, was 7 cases per 232 591 patients during 9 years. Our data confirm the results of previous investigations as regards the aggressive course and the need for aggressive treatment of WG as well as high mortality rate in this disease (28.5% in our patients). Of interest is that we noted relatively short time periods from disease onset to the establishment of diagnosis.