Department of Rheumatology, Institute of Medical Sciences, Uppsala University, Uppsala, Sweden.
J Rheumatol. 2010 Dec;37(12):2553-8. doi: 10.3899/jrheum.091280. Epub 2010 Oct 1.
The etiology of Wegener's granulomatosis (WG) is unknown. Susceptibility genes for WG that also affect the risks of other autoimmune/inflammatory diseases have been identified, indicating the existence of shared interdisease genetic susceptibilities. To determine the effect, on a population level, of shared susceptibility on disease risk, we assessed the occurrence of autoimmune/inflammatory disease in first-degree relatives of patients with WG.
In the Swedish Hospital Discharge Register we identified 2288 individuals discharged with the diagnosis of WG between 1970 and 2003. Through linkage to the Swedish Multi-generation Register we identified 787 parents, 1212 siblings, and 3650 children of these patients. From the Register of Total Population we identified 10 controls for each patient with WG, and 65,000 of their first-degree relatives. Through linkage to the nationwide Outpatients Register, we identified autoimmune/inflammatory disease among all relatives. Relative risks were estimated as hazard ratio (HR) using Cox regression. The study period was 2001-2006.
Biological first-degree relatives of patients with WG were at a moderately increased risk of any autoimmune/inflammatory disease (HR 1.32, 95% CI 1.18-1.49), including specific associations with, for example, multiple sclerosis (HR 1.92, 95% CI 1.16-3.16), Sjögren's syndrome (HR 2.00, 95% CI 1.07-3.73), and seropositive rheumatoid arthritis (HR 1.54, 95% CI 1.09-2.19).
Relatives of patients with WG are at increased risk of being diagnosed with other autoimmune/inflammatory diseases, indicating shared susceptibility between WG and other auto-immune/inflammatory disease.
韦格纳肉芽肿（WG）的病因尚不清楚。已经确定了影响其他自身免疫性/炎症性疾病风险的 WG 易感基因，这表明存在共享的疾病遗传易感性。为了确定在人群水平上，共享易感性对疾病风险的影响，我们评估了 WG 患者一级亲属中自身免疫性/炎症性疾病的发生情况。
我们在瑞典住院患者登记处中确定了 2288 名在 1970 年至 2003 年间被诊断为 WG 的患者。通过与瑞典多代登记处的链接，我们确定了这些患者的 787 名父母、1212 名兄弟姐妹和 3650 名子女。从总人口登记处，我们为每个 WG 患者确定了 10 名对照者，以及 65000 名他们的一级亲属。通过与全国门诊患者登记处的链接，我们确定了所有亲属中的自身免疫性/炎症性疾病。使用 Cox 回归估计相对风险作为危险比（HR）。研究期间为 2001-2006 年。
WG 患者的生物学一级亲属患任何自身免疫性/炎症性疾病的风险适度增加（HR 1.32，95%CI 1.18-1.49），包括与多发性硬化症（HR 1.92，95%CI 1.16-3.16）、干燥综合征（HR 2.00，95%CI 1.07-3.73）和血清阳性类风湿关节炎（HR 1.54，95%CI 1.09-2.19）的特定关联。
WG 患者的亲属患其他自身免疫性/炎症性疾病的风险增加，表明 WG 与其他自身免疫性/炎症性疾病之间存在共享的易感性。
