Medical Department I, Charité - University Medicine Berlin, Campus Benjamin Franklin, Hindenburgdamm 30, 12203 Berlin, Germany.
Eur J Clin Microbiol Infect Dis. 2011 Oct;30(10):1151-8. doi: 10.1007/s10096-011-1209-y. Epub 2011 Apr 2.
More than a century after its first description through G.H. Whipple, the understanding of the chronic multisystemic infection called Whipple's disease is still limited. However, within recent years the knowledge about diagnosis and treatment, the pathogenesis, and the biology of the agent itself have been improved by molecular biological and immunological methods. Despite the ubiquitous presence of the causative bacterium Tropheryma whipplei, Whipple's disease is very rare, and immunogenetic host factors rather than the genotype of the agent influence the course of infection. Since the clinical features of classical Whipple's disease are non-specific and the spectrum of isolated organ-specific manifestations might be underestimated, diagnosis often still is a challenge. Moreover, besides classical Whipple's disease, there are newly recognized infections with T. whipplei, which do not fit in the concept of classical Whipple's disease, for example, acute self-limiting infection and isolated T. whipplei endocarditis. Antibiotic therapy is usually successful. However, several problems are still unresolved, of which the most important are the following: which antibiotic should be used; how long treatment should be continued; how the immunoreconstitution inflammatory syndrome, which may occur after initiation of treatment, should be managed; and which is the best treatment of severe neurological manifestations.
在其首次被 G.H. Whipple 描述一个多世纪后，人们对这种称为 Whipple 病的慢性多系统感染的认识仍然有限。然而，近年来，通过分子生物学和免疫学方法，人们对该病的诊断和治疗、发病机制以及病原体本身的生物学有了更多的了解。尽管引起感染的 Tropheryma whipplei 无处不在，但 Whipple 病非常罕见，感染的进程受免疫遗传宿主因素的影响，而非病原体的基因型。由于经典 Whipple 病的临床特征不具有特异性，且孤立的器官特异性表现的范围可能被低估，因此诊断通常仍然具有挑战性。此外，除了经典 Whipple 病，还有新发现的 Tropheryma whipplei 感染，这些感染不符合经典 Whipple 病的概念，例如急性自限性感染和孤立的 Tropheryma whipplei 心内膜炎。抗生素治疗通常是有效的。然而，仍有几个问题尚未解决，其中最重要的是：应该使用哪种抗生素；治疗应该持续多久；在开始治疗后可能发生的免疫重建炎症综合征应如何处理；以及严重神经表现的最佳治疗方法是什么。
