儿童人群中的高丙种球蛋白血症作为潜在自身免疫性疾病的标志物：一项回顾性队列研究。
Hypergammaglobulinemia in the pediatric population as a marker for underlying autoimmune disease: a retrospective cohort study.
机构信息
Division of Immunology, Department of Medicine, Boston Children's Hospital and Department of Pediatrics, Harvard Medical School, 300 Longwood Avenue, Fegan 6, Boston, MA 02115, USA.
出版信息
Pediatr Rheumatol Online J. 2013 Nov 1;11(1):42. doi: 10.1186/1546-0096-11-42.
BACKGROUND
The significance of hypergammaglobulinemia as a marker of immune activation is unknown, as a differential diagnosis for hypergammaglobulinemia in children has not been adequately established. The goal of this study was to identify conditions associated with hypergammaglobulinemia in children, with the hypothesis that elevated immunoglobulin levels may precede or predict the development of autoimmune conditions.
METHODS
We reviewed the medical records for all children with IgG level ≥2000 mg/dL treated at a tertiary care children's hospital from January 1, 2000 through December 31, 2009. We compared clinical and laboratory features of these patients, and developed an algorithm to predict the likelihood of underlying autoimmunity based on these characteristics.
RESULTS
After excluding children who had received IVIG, a total of 442 patients with hypergammaglobulinemia were identified. Of these, nearly half had autoimmune conditions, most frequently systemic lupus erythematosus and lupus-related disorders. Autoimmune gastrointestinal disorders such as inflammatory bowel disease were also common. Infectious diseases were the next largest category of diseases, followed with much less frequency by malignant, drug-related, and other conditions. In comparison with non-autoimmune conditions, patients with autoimmune disease had higher IgG levels, lower white blood cell counts, lower hemoglobin values, and lower C-reactive protein (CRP) levels. Multivariable logistic regression confirmed that CRP (P = 0.002), white blood cell count (P < 0.001), hemoglobin (P = 0.015), and female gender (P < 0.001) are independent risk factors for autoimmune disease in patients with high IgG levels.
CONCLUSIONS
In a cohort of pediatric patients at a tertiary care children's hospital, hypergammaglobulinemia was most commonly associated with autoimmune diseases. In female patients with hypergammaglobulinemia, the presence of leukopenia, anemia, and normal CRP was 95% predictive of underlying autoimmune disease.
背景
高丙种球蛋白血症作为免疫激活的标志物的意义尚不清楚，因为儿童高丙种球蛋白血症的鉴别诊断尚未得到充分确立。本研究的目的是确定与儿童高丙种球蛋白血症相关的疾病，假设升高的免疫球蛋白水平可能先于或预测自身免疫性疾病的发生。
方法
我们回顾了 2000 年 1 月 1 日至 2009 年 12 月 31 日在一家三级儿童保健医院接受 IgG 水平≥2000mg/dL 治疗的所有儿童的病历。我们比较了这些患者的临床和实验室特征，并根据这些特征制定了一种预测潜在自身免疫可能性的算法。
结果
排除接受 IVIG 治疗的儿童后，共确定了 442 例高丙种球蛋白血症患儿。其中，近一半患有自身免疫性疾病，最常见的是系统性红斑狼疮和狼疮相关疾病。自身免疫性胃肠道疾病，如炎症性肠病也很常见。传染病是下一个最大的疾病类别，其次是恶性疾病、药物相关疾病和其他疾病。与非自身免疫性疾病相比，自身免疫性疾病患者的 IgG 水平更高，白细胞计数更低，血红蛋白值更低，C 反应蛋白（CRP）水平更低。多变量逻辑回归证实 CRP（P=0.002）、白细胞计数（P<0.001）、血红蛋白（P=0.015）和女性性别（P<0.001）是高 IgG 水平患者自身免疫性疾病的独立危险因素。
结论
在一家三级儿童保健医院的儿科患者队列中，高丙种球蛋白血症最常与自身免疫性疾病相关。在高丙种球蛋白血症的女性患者中，白细胞减少、贫血和正常 CRP 的存在对潜在自身免疫性疾病的预测准确率为 95%。
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