Clinical characteristics and long-term outcomes of 35 patients with Wegener's granulomatosis followed up at two rheumatology centers in Lithuania.
OBJECTIVE
The aim of this study was to investigate the survival of Lithuanian patients with Wegener's granulomatosis, who were followed up at two tertiary rheumatology centers, and to find the factors possibly influencing the outcomes of this disease.


METHODS
Thirty-five patients were followed up prospectively from the onset of disease (the first patient was enrolled in 1994) at Vilnius University Hospital and the Center of Rheumatology of Kaunas University of Medicine (17 and 18 patients, respectively). All patients in both the centers were followed up on a routine basis, and their records contained necessary information about laboratory and biopsy data; the censoring date (end of follow-up) was stated in June 2006.


RESULTS
Among the patients, the most frequent organs involved were ear, nose, throat (ENT) (82.6%), lungs (74.3%), and kidney (renal involvement was defined by proteinuria/abnormal urine sediment) (45.7%). Renal insufficiency was present in 20.6% of all the patients. At the end of the study, 32.4% of patients had simultaneously all three organ systems involved, namely upper respiratory tract, pulmonary, and renal. ANCA positivity was found for 26 (74.3%) of all the patients. Overall mortality rate was 25.7% (9/35). The mean survival was 99.4 months (95% CI, 73.6; 125.3) limited to 149 months for the longest-surviving patient.


CONCLUSIONS
Female gender and all three specific organ involvements being present at the same time and higher vasculitis damage index were associated with poor outcome. Overall mortality rate was 25.7% (9/35) during the 12-year follow-up, and it is similar to the data from other European countries.
Dadoniene
Jolanta
J
Centre for Innovative Medicine, State Research Institute, 01102 Vilnius, Lithuania. jolanta.dadoniene@ekmi.vu.lt
Pileckyte
Margarita
M
Baranauskaite
Asta
A
Kirdaite
Gailute
G
D000328:Adult; D000368:Aged; D056648:Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / Q000175:diagnosis / Q000401:mortality / Q000473:pathology; D001706:Biopsy; D002423:Cause of Death; D016009:Chi-Square Distribution; D005260:Female; D005500:Follow-Up Studies; D014890:Granulomatosis with Polyangiitis / Q000175:diagnosis / Q000401:mortality* / Q000473:pathology; D006801:Humans; D053208:Kaplan-Meier Estimate; D007680:Kidney Neoplasms / Q000175:diagnosis / Q000401:mortality* / Q000473:pathology; D008097:Lithuania; D016015:Logistic Models; D008175:Lung Neoplasms / Q000175:diagnosis / Q000401:mortality* / Q000473:pathology; D008297:Male; D008875:Middle Aged; D010039:Otorhinolaryngologic Neoplasms / Q000175:diagnosis / Q000401:mortality* / Q000473:pathology; D017063:Outcome Assessment, Health Care; D011446:Prospective Studies; D018709:Statistics, Nonparametric; D016019:Survival Analysis; D013997:Time Factors