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UCD 200系鸡的抗核抗体谱:一种进行性系统性硬化症模型

Antinuclear antibody profile in UCD line 200 chickens: a model for progressive systemic sclerosis.

作者信息

Gruschwitz M S, Shoenfeld Y, Krupp M, Gershwin M E, Penner E, Brezinschek H P, Wick G

机构信息

Institute for General and Experimental Pathology, University of Innsbruck Medical School, Austria.

出版信息

Int Arch Allergy Immunol. 1993;100(4):307-13. doi: 10.1159/000236430.

Abstract

The fully blown disease of human progressive systemic sclerosis (PSS, scleroderma) is serologically associated with the emergence of several types of autoantibodies, some of them regarded as more specific for scleroderma (e.g. Scl-70, anti-centromere) and some common also to other connective tissue diseases (e.g. anti-ssDNA). Since most patients suffering from PSS are not under medical control until clinical manifestations are fully established, only scarce data are available on the dynamics and clinical significance of autoantibodies in the very early stages of this systemic fibrotic disease. The University of California at Davis line 200 (UCD 200) of chickens spontaneously develop a PSS-like disorder with an acute inflammatory stage around 60 days after hatching, leading to fibrosis with fast progression. In order to address a possible correlation between the occurrence and titer of autoantibodies and the initial disease activity, we have chronologically investigated the presence and titer of autoantibodies directed against several human nuclear antigens in this animal strain. In UCD-200 chickens, we found a progressive increase in autoantibodies to histones, to ssDNA and--to a lesser degree--dsDNA with peaks at the age of 60 and 120 days, to poly(I) and poly(G) with a peak at 120 days and an elevation in anti-cardiolipin antibodies. Total immunoglobulin concentrations, anti-Ro, anti-La and anti-Sm showed no significant differences as compared to negative results in healthy normal controls. Our data reveal parallels in the antinuclear antibody (ANA) spectrum between UCD-200 chickens and human autoimmune collagen diseases, but do not reflect the typical ANA spectrum found in the foudroyant form of diffuse scleroderma.(ABSTRACT TRUNCATED AT 250 WORDS)

摘要

人类进行性系统性硬化症(PSS,硬皮病)的典型病症在血清学上与多种自身抗体的出现相关,其中一些被认为对硬皮病更具特异性(如Scl - 70、抗着丝粒抗体),还有一些在其他结缔组织疾病中也常见(如抗单链DNA抗体)。由于大多数PSS患者在临床表现完全显现之前未接受医学监测,关于这种系统性纤维化疾病早期自身抗体的动态变化及其临床意义的可用数据很少。加利福尼亚大学戴维斯分校的200系(UCD 200)鸡在孵化后约60天会自发出现类似PSS的病症,并伴有急性炎症阶段,随后迅速进展为纤维化。为了研究自身抗体的出现、滴度与初始疾病活动之间可能存在的相关性,我们按时间顺序调查了该动物品系中针对几种人类核抗原的自身抗体的存在情况和滴度。在UCD - 200鸡中,我们发现抗组蛋白、抗单链DNA抗体以及程度稍轻的抗双链DNA抗体呈逐渐增加趋势,在60天和120天时达到峰值;抗聚肌苷酸和抗聚鸟苷酸抗体在120天时达到峰值,抗心磷脂抗体水平升高。与健康正常对照的阴性结果相比,总免疫球蛋白浓度、抗Ro、抗La和抗Sm无显著差异。我们的数据揭示了UCD - 200鸡与人类自身免疫性胶原疾病在抗核抗体(ANA)谱方面的相似性,但未反映出弥漫性硬皮病暴发型中典型的ANA谱。(摘要截选至250词)

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