Objective: To explore age-based clinical and immune parameters in Henoch-Schönlein purpura (HSP) to determine clinically useful markers reflecting disease characteristic. Methods: A cohort of 502 patients with HSP were enrolled into this retrospective study to evaluate their clinical and immune data. Results: Majority HSP cases occurred at age ≤14 years and showed significant immune imbalances of ESR, CD3(+) cells, CD4(+) cells, CD3(-)CD16(+)CD56(+) cells, CD4(+)/CD8(+) cells, IgG, IgA, IgM, IgE, complements C3/C4 and ASO in the acute phase. Compared to patients aged >14 years, symptoms of joint were more frequent at disease onset in patients aged ≤14 years (20.8% vs 7.6%, χ(2)=13.547, P<0.001) , and involvement of digestive tract and joint were also more frequent (57.4% vs 33.8%, χ(2)=24.106, P<0.001; 55.9% vs 32.5%, χ(2)=23.768, P<0.001, respectively) , but not for involvement of kidney (21.4% vs 51.3%, χ(2)=42.440, P<0.001) . The patients aged ≤14 years had distinct immune state, reductions of CD3(+) cells, CD4(+) cells and IgG were more frequent than patients aged >14 years, also increase of ASO (33.1% vs 20.0%, χ(2)=6.656, P=0.010) , but not increase of IgA (2.6% vs 39.4%, χ(2)=15.582, P<0.001) . In addition, reduction of IgG and increase of IgE were positively associated with digestive tract involvement (P<0.001, P=0.001, respectively) , reduction of CD3(+)CD4(+) cells and normal IgM were positively associated with joint involvement (P=0.004, P=0.003, respectively) , increase of CD3(+)CD8(+) cells and normal CD3(+) cells were positively associated with kidney involvement (P=0.032, P=0.014, respectively) . Conclusion: HSP showed significant immune imbalance in the acute phase, patients between aged ≤14 and >14 years had distinct clinical and immune characteristic, and abnormal immune parameters were significantly associated with organ involvements.
目的：分析不同年龄亨-舒综合征（HSP）患者临床及免疫学特征，并探讨免疫指标与器官累及的相关性。 方法：回顾性分析502例HSP患者临床及免疫学特征，比较不同年龄组临床和免疫学特征的差异。 结果： 502例患者中345例（68.73%）首发年龄≤14岁。HSP患者急性期免疫紊乱主要表现为：红细胞沉降率（ESR）增快（41.5%，146/352）、CD3(+)细胞比例降低（62.9%，151/240）、CD4(+)细胞比例降低（48.3%，116/240）、CD4(+)/CD8(+)细胞比例升高（24.2%，58/240）、CD3(-)CD16(+)CD56(+)细胞比例失衡（21.7%，51/235）、IgA升高（19.7%，14/71）、IgG降低（54.9%，50/91）、IgM异常（13.6%，8/59）、IgE异常（60.2%，239/397）、补体C3降低（27.0%，17/63）、补体C4降低（7.1%，4/56）和抗链球菌溶血素O（ASO）升高（29.1%，111/381）。较之>14岁组患者，≤14岁组患者首发症状累及关节更为常见（20.8%对7.6%，χ(2)=13.547，P<0.001），消化道受累（57.4%对33.8%，χ(2)=24.106，P<0.001）和关节受累（55.9%对32.5%，χ(2)=23.768，P<0.001）更常见，而肾脏受累较少（21.4%对51.3%，χ(2)=42.440，P<0.001）；更多呈现CD3(+)、CD4(+)细胞比例降低，IgG降低和ASO升高（33.1%对20.0%，χ(2)=6.656，P=0.010），而IgA升高相对少见（2.6%对39.4%，χ(2)=15.582，P<0.001）。上述异常免疫学指标与HSP器官累及存在相关性，IgG水平降低（P<0.001）和IgE水平升高（P=0.001）的患者常累及消化道，CD3(+)CD4(+)细胞比例降低（P=0.004）和IgM水平正常（P=0.003）的患者倾向累及关节，而CD3(+)细胞比例正常（P=0.014）和CD3(+)CD8(+)细胞比例升高（P=0.032）的患者肾脏累及较为多见。 结论： HSP患者急性期存在显著的免疫紊乱，>14岁与≤14岁组间呈现不同的临床和免疫学异常特征，HSP器官累及与异常免疫学指标存在相关性。.
Keywords: Complement C3; Complement C4; Immunoglobulin; Lymphocyte subset; Purpura, Schoenlein-Henoch.