Lassalle P, Gosset P, Gruart V, Prin L, Capron M, Lagrue G, Kusnierz J P, Tonnel A B, Capron A
Centre d'Immunologie et de Biologie Parasitaire, Unité Mixte INSERM U 167, CNRS 624, Institut Pasteur, Lille, France.
Clin Exp Immunol. 1990 Oct;82(1):38-43. doi: 10.1111/j.1365-2249.1990.tb05400.x.
We reported three additional cases of a newly described syndrome called episodic angioedema with hypereosinophilia. In order to investigate its pathophysiological mechanisms, four parameters were concurrently investigated, including blood eosinophil density, serum chemoattractant activity, serum major basic protein (MBP) levels and the presence of anti-endothelial cell antibodies. Distribution of eosinophils through a metrizamide density gradient showed a preferential sedimentation of blood eosinophils in intermediate layers, clearly different from the hypodense cells (low-density layers) identified in a group of seven patients with idiopathic hypereosinophilic syndrome (HES). In two of the three patients with cyclic angioedema, a chemotactic activity towards eosinophils was detected in the serum (30 +/- 6 and 42 +/- 12 eosinophils per high-power field; P less than 0.05 compared with a control group). Serum MBP levels were at 1524, 619 and 1200 pg/ml. All three patients had circulating anti-endothelial cell antibodies, predominantly of the IgG isotype, in contrast to controls (P less than 0.01) or to patients with HES (P less than 0.01). Specificity of the antibody for endothelial cells was demonstrated in the three patients studied by the absence of binding to various blood cells, including monocytes, lymphocytes, eosinophils and platelets. In one case (patient 2), the levels of anti-endothelial cell antibodies, as well as the serum chemoattractant activity to eosinophils varied according to the successive acute phases of the disease. Although further investigations are needed to clarify the exact pathophysiology of this syndrome, and especially the possible participation of the anti-endothelial cell antibodies in the cutaneous lesions, these data suggest that angioedema observed in this syndrome could result from the combined effects of activated eosinophils and of immunologically induced endothelial lesions.
我们报告了另外三例一种新描述的综合征，即伴有嗜酸性粒细胞增多的发作性血管性水肿。为了研究其病理生理机制，同时对四个参数进行了研究，包括血液嗜酸性粒细胞密度、血清趋化活性、血清主要碱性蛋白（MBP）水平以及抗内皮细胞抗体的存在情况。通过甲泛葡胺密度梯度对嗜酸性粒细胞的分布进行分析，结果显示血液嗜酸性粒细胞优先沉积于中间层，这与一组7例特发性嗜酸性粒细胞增多综合征（HES）患者中所发现的低密度细胞（低密度层）明显不同。在三例周期性血管性水肿患者中的两例，血清中检测到对嗜酸性粒细胞的趋化活性（每高倍视野分别为30±6和42±12个嗜酸性粒细胞；与对照组相比，P<0.05）。血清MBP水平分别为1524、619和1200 pg/ml。与对照组（P<0.01）或HES患者（P<0.01）相比，所有三例患者均有循环抗内皮细胞抗体，主要为IgG同种型。在研究的三例患者中，通过未与包括单核细胞、淋巴细胞、嗜酸性粒细胞和血小板在内的各种血细胞结合，证实了该抗体对内皮细胞的特异性。在一例患者（患者2）中，抗内皮细胞抗体水平以及血清对嗜酸性粒细胞的趋化活性随疾病的连续急性期而变化。尽管需要进一步研究以阐明该综合征的确切病理生理学，尤其是抗内皮细胞抗体在皮肤病变中可能的作用，但这些数据表明，该综合征中观察到的血管性水肿可能是活化的嗜酸性粒细胞和免疫诱导的内皮病变共同作用的结果。
