Sluszniak M, Tarner I H, Thiele A, Schmeiser T
Klinik für Rheumatologie, Immunologie und Osteologie, Krankenhaus St. Josef Wuppertal, Bergstr. 6-12, 42105, Wuppertal, Deutschland.
Abt. für Rheumatologie, Klinische Immunologie, Osteologie und Physikalische Medizin, Kerckhoff-Klinik GmbH Bad Nauheim, Bad Nauheim, Deutschland.
Z Rheumatol. 2019 Feb;78(1):55-65. doi: 10.1007/s00393-018-0573-8.
Whipple's disease (WD) is a rare, chronic multiorgan disease which can caused by Tropheryma whipplei, a ubiquitous gram positive bacterium. Detection of T. whipplei is mostly performed histologically using periodic acid-Schiff (PAS) staining in affected tissues to visualize characteristic PAS-positive macrophages and by the polymerase chain reaction (PCR). Clinically, WD is often characterized by gastrointestinal symptoms (diarrhea, colic-like abdominal pain and weight loss). Arthritis is a common presentation of WS, often leading to a misdiagnosis of seronegative rheumatoid arthritis and as a consequence to immunosuppressive therapy. The clinical presentation of WD is highly polymorphic affecting different organ systems (e. g. cardiac or neurological manifestation) and making an appropriate clinical diagnosis and even the diagnostic process itself difficult. This article reports on three cases presenting with completely different leading symptoms (initially misdiagnosed as seronegative rheumatoid arthritis, spondyloarthritis and adult onset of Still's disease, respectively) that illustrate the rich diversity of WD. The cases were chosen to draw attention to the fact that although WD is mainly associated with the field of gastroenterology and gastrointestinal (GI) involvement is common, it may appear without GI symptoms. In cases of a clinical suspicion of WD, diagnostic efforts should be made to detect the bacterium in the affected organ. The German S2k guidelines on GI infections and WD published in January 2015 summarized the current state of the art for WD. The currently recommended primary treatment is antibiotics that can infiltrate the cerebrospinal fluid, e. g. ceftriaxone, followed by cotrimoxazole, which should be maintained over several months.
惠普尔病(WD)是一种罕见的慢性多器官疾病,由普遍存在的革兰氏阳性菌 Tropheryma whipplei 引起。检测 Tropheryma whipplei 主要通过组织学方法,在受影响的组织中使用过碘酸希夫(PAS)染色来观察特征性的 PAS 阳性巨噬细胞,以及通过聚合酶链反应(PCR)进行。临床上,WD 通常以胃肠道症状(腹泻、绞痛样腹痛和体重减轻)为特征。关节炎是 WD 的常见表现,常导致血清阴性类风湿关节炎的误诊,并因此接受免疫抑制治疗。WD 的临床表现高度多样,可影响不同的器官系统(如心脏或神经表现),使得做出恰当的临床诊断甚至诊断过程本身都很困难。本文报道了三例具有完全不同主要症状的病例(最初分别误诊为血清阴性类风湿关节炎、脊柱关节炎和成人斯蒂尔病),这些病例说明了 WD 的丰富多样性。选择这些病例是为了提醒人们注意,尽管 WD 主要与胃肠病学领域相关且胃肠道受累很常见,但它也可能在没有胃肠道症状的情况下出现。在临床怀疑 WD 的病例中,应努力在受影响的器官中检测该细菌。2015 年 1 月发布的德国关于胃肠道感染和 WD 的 S2k 指南总结了 WD 的当前技术水平。目前推荐的主要治疗方法是使用能够渗入脑脊液的抗生素,例如头孢曲松,随后使用复方新诺明,治疗应持续数月。