Schultz D R, Perez G O, Volanakis J E, Pardo V, Moss S H
Am J Kidney Dis. 1981 Nov;1(3):157-65. doi: 10.1016/s0272-6386(81)80022-4.
We assessed the clinical, serologic, and renal abnormalities of two patients with the syndrome of hypocomplementemia, vasculitis, and urticaria. Both patients presented with recurrent urticaria and were found subsequently to have glomerulonephritis. Both manifested depletion of the early complement (C) components, and their Cl inhibitor levels were normal. Circulating immune complexes were detected by two of three assays in both patients. One patient had a high titer of Sm antibodies. The detectable immune complexes were of high molecular weight, and were more suggestive of classical lupus erythematosus than the low molecular weight (7S) Clq precipitins which characterize some cases of hypocomplementemia-vasculitis-urticaria syndrome. Biopsy of the skin lesions in both patients revealed leukocytoclastic vasculitis with immunoglobulin and C deposits at the dermal-epidermal junction. Renal biopsy specimens showed segmental or diffuse proliferative mesangial glomerulonephritis. The results suggest that patients with this syndrome do not conform to a discrete entity but rather exhibit a spectrum of diseases ranging from systemic lupus erythematosus to the syndrome of urticaria, hypocomplementemia, and low molecular weight Clq precipitins.
我们评估了两名患有低补体血症、血管炎和荨麻疹综合征患者的临床、血清学及肾脏异常情况。两名患者均表现为复发性荨麻疹，随后被发现患有肾小球肾炎。二者均表现出早期补体（C）成分的消耗，且其C1抑制物水平正常。两名患者的三种检测方法中有两种检测到循环免疫复合物。一名患者抗Sm抗体滴度较高。可检测到的免疫复合物为高分子量，相较于一些低补体血症-血管炎-荨麻疹综合征病例所特有的低分子量（7S）C1q沉淀素，更提示为典型的红斑狼疮。两名患者皮肤病变活检均显示白细胞破碎性血管炎，在真皮-表皮交界处有免疫球蛋白和补体沉积。肾活检标本显示节段性或弥漫性增生性系膜肾小球肾炎。结果表明，该综合征患者并不符合一个独立的疾病实体，而是表现出一系列疾病，范围从系统性红斑狼疮到荨麻疹、低补体血症和低分子量C1q沉淀素综合征。
