Environmental risk factors in endemic pemphigus foliaceus (Fogo selvagem)

Environmental risk factors in endemic pemphigus foliaceus (Fogo selvagem)
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DOI:
10.1111/j.1087-0024.2004.00833.x
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发表时间:
2004-01-01
影响因子:
--
通讯作者:
Diaz, LA
Diaz, LA
中科院分区:
其他
文献类型:
--
作者:
Aoki, V;Millikan, RC;Diaz, LA

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Limao Verde的Terena保留区正在进行的血清流行病学研究,已知FS的患病率和发病率很高,揭示了有关这种自身免疫性疾病的重要信息。在1994年开始的对大约1,200人的监测中,我们记录了43例FS病例,并研究了其中几个人从正常状态到疾病状态的转变。此外,我们确定FS患者以及大量的正常人保留具有抗dsg 1自身抗体。结果表明:(1)dsg_1的胞外域含有FS患者自身抗体和T细胞识别的表位,(2)FS患者的致病性抗dsg_1自身抗体属于IgG_4亚类,(3)在Limao Verde的正常人和临床前患者中检测到IgG_1亚类的非致病性抗dsg_1自身抗体,(4)在FS患者中检测到IgG_1亚类的非致病性抗dsg_1自身抗体。(4)正常人和临床前期FS患者的抗dsg 1自身抗体识别dsg 1的EC 5结构域,而致病性抗dsg 1自身抗体结合EC 1/EC 2结构域;(5)FS患者的房屋是乡村风格的,屋顶和墙壁是茅草,地板是泥土;(6)吸血昆虫的出现频率较高(臭虫和接吻虫)在FS患者的房屋中;(7)先前的研究表明,该保护区的主要黑蝇属于黑拟黑蝇。这些发现表明,触发FS自身免疫反应的环境抗原可能与暴露于吸血昆虫有关。
An ongoing sero-epidemiological study of the Terena reservation of Limao Verde, known to have a high prevalence and incidence of FS, has revealed important information about this autoimmune disease. During surveillance of this population of approximately 1,200, which began in 1994, we documented 43 FS cases and studied the transition from the normal state to the disease state in several of these individuals. Furthermore, we established that FS patients as well as a large number of normal individuals on the reservation possess anti-dsg1 autoantibodies. The following interesting observations were made: (1) the ectodomain of dsg1 contains epitopes recognized by both autoantibodies and T cells from FS patients; (2) pathogenic anti-dsg1 autoantibodies in FS belong to the IgG4 subclass; (3) nonpathogenic anti-dsg1 autoantibodies of the IgG1 subclass were detected in normal individuals from Limao Verde and in patients in the preclinical stage of the disease; (4) anti-dsg1 autoantibodies from normal individuals and patients in the preclinical stage of FS recognize the EC5 domain of dsg1, whereas pathogenic anti-dsg1 autoantibodies bind the EC1/EC2 domains; (5) houses of FS patients are rustic, with thatched roofs and walls and dirt floors; (6) there was a high frequency of hematophagous insects (bedbugs and kissing bugs) in the houses of FS patients; (7) previous studies revealed that the predominant black fly on this reservation belongs to the species Simunlium nigrimanum. These findings suggest that the environmental antigen(s) triggering the autoimmune response in FS may be linked to exposure to hematophagous insects.