Schneider Thomas, Moos Verena, Loddenkemper Christoph, Marth Thomas, Fenollar Florence, Raoult Didier
Medical Department I, Charité-University Medicine Berlin, Campus Benjamin Franklin, Berlin, Germany.
Lancet Infect Dis. 2008 Mar;8(3):179-90. doi: 10.1016/S1473-3099(08)70042-2.
100 years after its first description by George H Whipple, the diagnosis and treatment of Whipple's disease is still a subject of controversy. Whipple's disease is a chronic multisystemic disease. The infection is very rare, although the causative bacterium, Tropheryma whipplei, is ubiquitously present in the environment. We review the epidemiology of Whipple's disease and the recent progress made in the understanding of its pathogenesis and the biology of its agent. The clinical features of Whipple's disease are non-specific and sensitive diagnostic methods such as PCR with sequencing of the amplification products and immunohistochemistry to detect T whipplei are still not widely distributed. The best course of treatment is not completely defined, especially in relapsing disease, neurological manifestations, and in cases of immunoreconstitution after initiation of antibiotic treatment. Patients without the classic symptoms of gastrointestinal disease might be misdiagnosed or insufficiently treated, resulting in a potentially fatal outcome or irreversible neurological damage. Thus, we suggest procedures for the improvement of diagnosis and an optimum therapeutic strategy.
在乔治·H·惠普尔首次描述惠普尔病100年后，其诊断和治疗仍然是一个有争议的话题。惠普尔病是一种慢性多系统疾病。尽管致病细菌惠普尔嗜组织菌在环境中普遍存在，但这种感染非常罕见。我们回顾了惠普尔病的流行病学以及在其发病机制和病原体生物学理解方面取得的最新进展。惠普尔病的临床特征不具有特异性，而诸如对扩增产物进行测序的聚合酶链反应（PCR）和检测惠普尔嗜组织菌的免疫组织化学等敏感诊断方法仍未广泛应用。最佳治疗方案尚未完全确定，尤其是在复发疾病、神经表现以及抗生素治疗开始后的免疫重建病例中。没有胃肠道疾病典型症状的患者可能会被误诊或治疗不充分，从而导致潜在的致命后果或不可逆转的神经损伤。因此，我们提出了改善诊断的程序和最佳治疗策略。
