West C D, McAdams A J, Welch T R
Children's Hospital Research Foundation, Cincinnati, OH 45229-3039.
Pediatr Nephrol. 1994 Dec;8(6):677-83. doi: 10.1007/BF00869088.
Ten patients with Henoch-Schöenlein purpura (HSP) were selected for study because their early renal biopsies showed focal and segmental hypercellularity, with IgA present only in deposits at the periphery of the lobules. Mesangial deposits of IgA were absent. All had laboratory evidence of nephrotic syndrome and/or renal compromise. The glomerular hypercellularity was largely the result of the infiltration of monocytes whose cytoplasm often contained tubular lysosomes and wrapping lysosomal membranes, evidence of monocyte activation. Mean levels of C3 were normal but those of C4 and properdin significantly depressed. This complement profile, as well as a glomerular monocytic infiltrate, are also seen in essential cryoglobulinemia in the adult. Of follow-up biopsies in six patients, the glomeruli were normal in three, with no IgA deposition. In the other three, mesangial deposits of IgA typical of HSP were present. The initial focal-segmental glomerulitis of these patients appeared to be the benign first phase of a disease which had the potential to culminate in mesangial IgA deposition. Patients like the three who escaped mesangial IgA would be among those responsible for the observed dissociation between severity of the initial illness and ultimate prognosis.
选择10例过敏性紫癜（HSP）患者进行研究，因为他们早期的肾活检显示局灶性和节段性细胞增多，IgA仅存在于小叶周边的沉积物中。系膜IgA沉积物缺失。所有患者均有肾病综合征和/或肾功能损害的实验室证据。肾小球细胞增多主要是单核细胞浸润的结果，其细胞质中常含有管状溶酶体和包裹的溶酶体膜，这是单核细胞活化的证据。C3平均水平正常，但C4和备解素水平显著降低。这种补体谱以及肾小球单核细胞浸润在成人原发性冷球蛋白血症中也可见到。在6例患者的随访活检中，3例肾小球正常，无IgA沉积。另外3例存在HSP典型的系膜IgA沉积。这些患者最初的局灶节段性肾小球肾炎似乎是一种疾病的良性第一阶段，这种疾病有可能最终导致系膜IgA沉积。像这3例未出现系膜IgA沉积的患者可能是导致观察到的初始疾病严重程度与最终预后之间脱节的原因之一。
