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Our aim was to describe the treatment and survival characteristics of Wegener's granulomatosis (WG) in Canterbury, New Zealand. The medical records of 48 patients diagnosed between 1 July 1997 and 31 December 2008 and fulfilling validated classification criteria for WG were reviewed to characterise survivorship in the province of Canterbury, New Zealand. The age at diagnosis was 61 years (range 20 to 83 years) with an equal number of males and females. Using Kaplan-Meier product-limit analysis, the probability of survival at 1 and 10 years was 91% and 62% respectively. Of the 12 deaths in the cohort, four occurred within 12 months of diagnosis: two from pulmonary haemorrhage and two from renal failure. Beyond 12 months, two patients died of renal failure, two of myocardial infarction and one from cardiac arrhythmia, one from cerebrovascular disease and two from colorectal carcinoma. The median time to relapse was 6.75 years, and the probability of relapse within 10 years was 67%. Survivorship, treatment response rate and the rate of relapse from WG in a cohort of patients from this high-prevalence southern hemisphere region were similar to that reported for northern hemisphere cohorts with a similar prevalence. [ABSTRACT FROM AUTHOR]

Background: The epidemiology of Wegener’s granulomatosis (WG) has shown a latitude-dependent predisposition in Northern Hemisphere and in New Zealand. There are no studies describing epidemiology or long-term follow up of WG reported from Australia. The aims of this study were to describe the epidemiological and clinical features of WG in South Australia (SA). Methods: The 5-year incidence of WG in SA over the period 2001–2005 was determined using the International Classification of Diseases classification (M313) of the discharge diagnosis using the Integrated South Australian Activity Collection. A retrospective case record analysis of 30 patients fulfilling the American College of Rheumatology criteria for WG and managed at two regional hospitals over a 20-year period (1985–2004) was carried out. Results: The 5-year incidence of WG in SA was 56 per 106 (95% confidence interval 44.1–68.4 per 106). There were no regional or seasonal variations in disease occurrence. The demographic, clinical and serological features in the clinical study were similar to previously published studies. Significant treatment-related morbidity was noted with 50% of patients having atherosclerotic vascular complications. The median survival was 12 years. There were two important periods with greater risk of dying – in the first 5 years and after 10 years. Conclusion: The 5-year incidence of WG in SA is higher than that in the same latitudinal region in New Zealand (∼34°S). Atherosclerotic vascular disease was a major long-term morbidity. There is increased incidence of early mortality, warranting the need for earlier diagnosis and better therapies. Further studies from Southern Hemisphere are required for better epidemiological description of this disease. [ABSTRACT FROM AUTHOR]

Background: The aim of the study was to determine whether there was evidence for a geographic gradient in the incidence of Wegener’s granulomatosis (WG) and WG-like disease in New Zealand (NZ). Methods: The National Minimum Dataset of the Ministry of Health, NZ was searched for individual patient discharges coded by the International Classification of Diseases 10th Revision, Australian Modification as either M301 (polyarteritis with lung involvement, including Churg Strauss and allergic granulomatous angiitis) or M313 (WG, necrotizing respiratory granulomatosis) for the period 1 January 1999 to 31 December 2003. Data were standardized using the 2001 NZ census. Results: One hundred and ninety-five patients (95 men) were given a first-time discharge code of either M301 (40 patients) or M313 (155 patients). No gender bias was seen. The rate among Europeans was twice that of NZ Maoris or Asians. The rate of disease peaked in the age band 70–79 years and during winter months. A significant positive north–south geographic gradient was present for M313. No difference in the rate of readmission or time to relapse between geographic regions was found for M313. Conclusion: A north–south gradient in the rate of patient discharges given a diagnostic code of M313 (WG, necrotizing respiratory granulomatosis) was present in NZ. This finding supports the hypothesis that there is a latitude-dependent risk factor(s) for WG possibly common to both global hemispheres. [ABSTRACT FROM AUTHOR]