不同年龄亨-舒综合征患者临床与免疫学特征研究

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.