Gibson A, Stamp L K, Chapman P T, O'Donnell J L
Department of Rheumatology, Immunology and Allergy, Canterbury District Health Board, University of Otago, Christchurch, New Zealand.
Rheumatology (Oxford). 2006 May;45(5):624-8. doi: 10.1093/rheumatology/kei259. Epub 2005 Dec 20.
To determine the prevalence of Wegener's granulomatosis (WG) and microscopic polyangiitis (MPA) in the province of Canterbury, New Zealand.
Three hospital clinical databases and the immunology laboratory database were searched and case notes reviewed for patients fulfilling either the 1990 American College of Rheumatology (ACR) criteria for WG or a modification of those criteria that allowed for antineutrophil cytoplasmic antibody (ANCA) positivity in the absence of granulomatous vasculitis. MPA was defined by the Chapel Hill consensus definition; however, in the absence of histological evidence of pauci-immune glomerulonephritis, ANCA positivity in association with evidence of active glomerular disease was included as a criterion. The point prevalence at 31 December 2003 and the 5-yr period prevalence for the interval 1 January 1999 to 31 December 2003 were calculated.
Seventy-three patients with WG and 28 patients with MPA fulfilled the inclusion criteria. A 5-yr period prevalence of 152 WG cases/million [95% confidence interval (CI) 117-186] and 58 MPA cases/million (95% CI 37-80) was calculated using 2001 census data as denominator. Nineteen patients with WG died and 10 patients with MPA died during the study period, resulting in a point prevalence for survivors at 31 December 2003 of 112 cases/million (95% CI 82-142) and 37 cases/million (95% CI 20-55), respectively. Using unmodified ACR criteria the 5-yr period and point prevalence for WG were 131/million (95% CI 99-163) and 93.5/million (95% CI 66-121), respectively. Apart from respiratory tract involvement, which formed part of the case definition of WG, organ involvement was similar in both diseases.
The prevalence of WG and MPA in Canterbury is the highest reported to date. Restricting the case definition of WG to the ACR classification criteria we found a prevalence equivalent to that described in northern Norway. The clinical severity and serological characteristics were similar to descriptions in other WG and MPA patient cohorts. Studies of disease prevalence in other Southern Hemisphere centres will determine if the observed north-south negative disease gradient in the Northern Hemisphere is reciprocated.
确定新西兰坎特伯雷地区韦格纳肉芽肿（WG）和显微镜下多血管炎（MPA）的患病率。
检索了三个医院临床数据库和免疫实验室数据库，并查阅了符合1990年美国风湿病学会（ACR）WG标准或对这些标准进行修改后允许在无肉芽肿性血管炎情况下抗中性粒细胞胞浆抗体（ANCA）阳性的患者病历。MPA由查珀尔希尔共识定义确定；然而，在缺乏寡免疫性肾小球肾炎组织学证据的情况下，将ANCA阳性与活动性肾小球疾病证据相关联作为一项标准。计算了2003年12月31日的时点患病率以及1999年1月1日至2003年12月31日这5年期间的患病率。
73例WG患者和28例MPA患者符合纳入标准。以2001年人口普查数据为分母，计算出5年期间WG患病率为152例/百万[95%置信区间（CI）117 - 186]，MPA患病率为58例/百万（95%CI 37 - 80）。在研究期间，19例WG患者死亡，10例MPA患者死亡，导致2003年12月31日幸存者的时点患病率分别为112例/百万（95%CI 82 - 142）和37例/百万（95%CI 20 - 55）。使用未修改的ACR标准，WG的5年期间患病率和时点患病率分别为131/百万（95%CI 99 - 163）和93.5/百万（95%CI 66 - 121）。除呼吸道受累（这是WG病例定义的一部分）外，两种疾病的器官受累情况相似。
坎特伯雷地区WG和MPA的患病率是迄今为止报道的最高值。将WG的病例定义限制在ACR分类标准内，我们发现其患病率与挪威北部描述的相当。临床严重程度和血清学特征与其他WG和MPA患者队列的描述相似。对其他南半球中心疾病患病率的研究将确定北半球观察到的南北疾病负梯度是否相反。
