Wegener's granulomatosis in Finland in 1981-2000: risk of dialysis-dependent renal disease.

OBJECTIVE: To determine the risk of renal insufficiency requiring transient or chronic dialysis and its contributing factors in patients diagnosed with Wegener's granulomatosis (WG) between 1981 and 2000 in Finland. PATIENTS AND METHODS: A retrospective cohort study using hospital discharge registers with a review of hospital case reports. Data were complemented with files from the Finnish Registry for Kidney Diseases. All files were reviewed by 8 October 2005. RESULTS: A total of 492 patients received a verified diagnosis of WG in 1981-2000. Of these, 84 (17%) needed dialysis by the end of follow-up. Of the 84 dialysed patients, 32 (38%) recovered initially, 32 (38%) needed chronic dialysis (dialysed > 3 months), and 19 (23%) received a kidney transplant. Forty-one (49%) of the dialysed patients were alive at the end of follow-up: 16 with a kidney transplant, 14 on dialysis, and 11 without dialysis. The cumulative rate of developing renal involvement leading to dialysis was 14.6% [95% confidence interval (CI) 11.6-18.2] at 5 years and 29.6% (95% CI 21.5-40.0) at 20 years after onset of WG symptoms. Elevated creatinine levels at presentation were associated with an increased rate of renal insufficiency requiring chronic dialysis. Age, gender, and involvement of any particular organ system had no significant effect. CONCLUSIONS: WG patients are at great risk of developing renal insufficiency, and this risk increases as the disease progresses. Elevated creatinine levels at presentation are associated with an increased risk of dialysis-dependent end-stage renal disease.

Survival of patients with Wegener's granulomatosis diagnosed in Finland in 1981-2000.

OBJECTIVE: To determine the survival of and contributing factors in patients diagnosed with Wegener's granulomatosis (WG) between 1981 and 2000 in Finland. METHODS: A retrospective cohort study using hospital discharge registers with a review of hospital case records. Analysis of causes of death in Finland up to 30 July 2005. RESULTS: A total of 492 patients received a verified diagnosis of WG between 1981 and 2000. Of these, 203 died before the end of June 2005. The overall 1-year survival rate was 83.3% and the 5-year survival rate was 74.2%. The standardized mortality ratio (SMR) for all WG patients was 3.43 [95% confidence interval (CI) 2.98-3.94], for women 4.38 (95% CI 3.59-5.61) and for men 2.80 (95% CI 2.28-3.41). The most frequent causes of death were WG or another connective tissue disease, cardiovascular events and neoplasms. The prognosis did not change markedly over the 20-year period. Older age and elevated creatinine level at presentation were associated with poorer prognosis, whereas primary ear, nose, and throat (ENT) involvement and prompt treatment with cyclophosphamide predicted longer survival. CONCLUSIONS: WG patients had increased mortality compared with the rest of the population. Older age and elevated creatinine level at diagnosis predicted poor prognosis, but ENT involvement initially and cyclophosphamide treatment resulted in a better outcome.

Incidence of Wegener's granulomatosis in Finland 1981-2000.

OBJECTIVE: To determine the incidence and clinical presentation of Wegener's granulomatosis (WG) in Finland during the 20-year period 1981-2000. METHODS: We performed a study with retrospective data retrieval using the hospital discharge register in Finland. All available hospital case reports were reviewed. We included those patients diagnosed with and treated for WG. Demographic and clinical data at diagnosis were recorded. RESULTS: Of the 492 patients diagnosed with WG, 49% were male. Mean age at diagnosis was 53.2 years (SD 18.1). The highest rate of incidence occurred in men and women aged 65-74 years. The annual incidence per million of the population increased from 1.9 (95% CI 1.4 to 2.6) during 1981-1985 to 9.3 (95% CI 8.1 to 10.6) during 1996-2000 with gender age-adjusted incidence rate ratio 4.5 (CI 3.6 to 5.7). Only minor changes in the signs and symptoms at diagnosis occurred during the 20-year span. In 83% of cases, the ACR criteria (>or=2 criteria) were fulfilled. The mean age at diagnosis rose from 45.8 to 55.0 years and the median diagnostic delay decreased from 17 to 4 months during the two decades. CONCLUSION: The incidence of WG has increased during the last two decades with little change in clinical symptoms at presentation. At the same time, the mean age of the patients has increased and the diagnostic delay has considerably shortened.

Wegener's granulomatosis in Finland in 1981-2000: clinical presentation and diagnostic delay.

OBJECTIVE: To determine the features of clinical presentation and the diagnostic delay of Wegener's granulomatosis (WG) in Finland in 1981-2000. METHODS: A retrospective cohort study using hospital discharge registers with review of hospital case reports. We determined the spectrum of WG symptoms in relation to individual organ groups and ELK (Ear, nose, throat, Lung, Kidney) score within the first 6 months from disease onset. Diagnostic delay and contributing factors were analysed. RESULTS: Of 513 WG patients treated at Finnish specialized medical care hospitals, 489 patients had sufficient information in the first 6 months of their disease. The ENT (Ear, Nose, and Throat) system was involved in 63%; the pulmonary system in 59%, and 60% had general symptoms. Over time, the general symptoms became more common at presentation. The diagnostic delay had decreased from 17 to 4 months. CONCLUSIONS: The initial symptoms of WG remained essentially unchanged. The proportion of general symptoms increased. A considerable shortening of diagnostic delay occurred, mostly between the 5-year periods of 1981-1985 and 1986-1990, when anti-neutrophil cytoplasmic antibody (ANCA) tests were brought into routine use. ELK scores of 2 and 3 and the presence of pulmonary and general symptoms predicted a shorter diagnostic delay.