Odendahl M, Keitzer R, Wahn U, Hiepe F, Radbruch A, Dörner T, Bunikowski R
Deutsches Rheuma-Forschungszentrum Berlin, Germany.
Ann Rheum Dis. 2003 Sep;62(9):851-8. doi: 10.1136/ard.62.9.851.
To investigate the distribution of peripheral B cell subpopulations of children with active and inactive systemic lupus erythematosus (SLE) compared with healthy controls.
Peripheral B cell subpopulations of 11 children with SLE (6 with active and 5 with inactive disease) and 14 age matched normal healthy children were analysed. Active disease was diagnosed in children with a flare of SLE, who received treatment by i.v. cyclophosphamide or i.v. methylprednisolone pulse to control the disease. Additionally, the peripheral B cells of the children with SLE were compared with those of 13 consecutive patients with adult onset SLE.
No major difference was found in the frequency and total number of CD27(-)/CD19(+) naïve B cells and CD27(+)/CD19(+) memory B cells between patients with active and inactive lupus and healthy controls, but there was a significant increase in CD27(high) expressing plasma blasts in patients with active SLE. These cells coexpress CD38(+), HLA-DR(dim), surface Ig(low) and lack the expression of CD20 but are clearly positive for intracellular Ig, indicative of early plasma cells. Most CD138(+) cells coexpress CD27(high)/CD19(+). The enhanced frequency of peripheral plasma blasts in children with active SLE is consistent with previous findings in adult patients with SLE, whereas a relative predominance of CD27(+) memory B cells was only identified in the adult patients.
Profound abnormalities in the distribution of B cell compartments are more pronounced in older patients with SLE, but an enhanced frequency and cell number of peripheral plasma blasts is characteristic of both diseases during active stages. Thus detection of CD27(high) plasma blasts significantly correlates with active lupus in both children and adults.
研究活动期和非活动期系统性红斑狼疮（SLE）患儿外周血B细胞亚群的分布情况，并与健康对照进行比较。
分析了11例SLE患儿（6例活动期和5例非活动期）以及14例年龄匹配的正常健康儿童的外周血B细胞亚群。活动期疾病定义为SLE病情复发的患儿，这些患儿接受静脉注射环磷酰胺或静脉注射甲泼尼龙冲击治疗以控制病情。此外，还将SLE患儿的外周血B细胞与13例连续的成人起病SLE患者的外周血B细胞进行了比较。
活动期和非活动期狼疮患者与健康对照之间，CD27(-)/CD19(+)幼稚B细胞和CD27(+)/CD19(+)记忆B细胞的频率和总数没有显著差异，但活动期SLE患者中表达CD27(高)的浆母细胞显著增加。这些细胞共表达CD38(+)、HLA-DR(低)、表面Ig(低)，且不表达CD20，但细胞内Ig呈明显阳性，提示为早期浆细胞。大多数CD138(+)细胞共表达CD27(高)/CD19(+)。活动期SLE患儿外周血浆母细胞频率增加与成年SLE患者先前的研究结果一致，而仅在成年患者中发现CD27(+)记忆B细胞相对占优势。
B细胞亚群分布的严重异常在老年SLE患者中更为明显，但外周血浆母细胞频率和细胞数量增加是两种疾病活动期的特征。因此，检测CD27(高)浆母细胞与儿童和成人的活动期狼疮显著相关。
