Berntson Lillemor, Damgård Michael, Andersson-Gäre Boel, Herlin Troels, Nielsen Susan, Nordal Ellen, Rygg Marite, Zak Marek, Fasth Anders
Department of Women's and Children's Health, Uppsala University Children's Hospital, Uppsala, Sweden.
J Rheumatol. 2008 Oct;35(10):2055-61. Epub 2008 Sep 1.
Juvenile idiopathic arthritis (JIA) is a heterogeneous condition with very few clinical and laboratory signs that can help predict the course and severity of the disease in the individual patient. The cell-surface antigen HLA-B27 is well known to be associated with spondyloarthropathies, reactive arthritis, and enthesitis. HLA-B27 plays an important role in the classification of JIA, since evidence of sacroiliitis most often evolves after years of arthritis in other joints. We investigated the associations of HLA-B27 and the clinical manifestations of JIA using a method as close to a population-based study as possible.
We studied an incidence-based cohort of 305 patients collected prospectively in 3 Nordic countries (Sweden, Norway, Denmark). Clinical and serological data of the first 3 years of the disease were collected.
HLA-B27 was found to be positive in 25.5% of the patients, and we found a higher proportion of HLA-B27-positive boys with older age at disease onset (p=0.034). Regression analysis showed a correlation of 0.7 in the HLA-B27-positive boys, pointing to a higher risk of more joint involvement with older age at disease onset. By Fisher's exact test, involvement of small joints in the lower extremities was associated with HLA-B27 in boys (p=0.011), but not in girls (p=0.687). HLA-B27 was associated with inflammatory back pain in both sexes (p=0.041 in boys, p=0.042 in girls), but with enthesitis only in boys (p<0.001 in boys, p=0.708 in girls).
HLA-B27 is of increasing importance with older age at disease onset in boys with JIA, predicting more active joints within the first 3 years of disease, and also involving small joints in the lower extremity to a greater degree than in HLA-B27-negative boys. During the first 3 years of disease the occurrence of HLA-B27 is associated with inflammatory back pain in both sexes, but with enthesitis only in boys. Our data present new challenges for the ILAR classification of JIA.
幼年特发性关节炎（JIA）是一种异质性疾病，几乎没有临床和实验室指标能够帮助预测个体患者的疾病进程和严重程度。细胞表面抗原HLA - B27与脊柱关节病、反应性关节炎和附着点炎密切相关。HLA - B27在JIA的分类中起着重要作用，因为骶髂关节炎的证据通常在其他关节出现关节炎数年之后才会出现。我们尽可能采用接近基于人群研究的方法，调查了HLA - B27与JIA临床表现之间的关联。
我们研究了在3个北欧国家（瑞典、挪威、丹麦）前瞻性收集的305例基于发病率的队列患者。收集了疾病前3年的临床和血清学数据。
发现25.5%的患者HLA - B27呈阳性，我们发现疾病发病时年龄较大的HLA - B27阳性男孩比例更高（p = 0.034）。回归分析显示HLA - B27阳性男孩的相关性为0.7，表明疾病发病时年龄越大，关节受累更多的风险越高。通过Fisher精确检验，下肢小关节受累在男孩中与HLA - B27相关（p = 0.011），而在女孩中不相关（p = 0.687）。HLA - B27在男女中均与炎性背痛相关（男孩中p = 0.041，女孩中p = 0.042），但仅在男孩中与附着点炎相关（男孩中p<0.001），女孩中p = 0.708）。
在发病时年龄较大的JIA男孩中，HLA - B27的重要性日益增加，它可预测疾病前3年内关节活动更为活跃，且与HLA - B27阴性男孩相比，下肢小关节受累程度更高。在疾病的前3年中，HLA - B27的出现与男女两性的炎性背痛相关，但仅与男孩的附着点炎相关。我们的数据为JIA的国际风湿病联盟（ILAR）分类提出了新的挑战。
