von Gerstenbergk C, Lorenz H-M, Blank N
Sektion Rheumatologie, Medizinische Klinik V, Universitätsklinikum Heidelberg.
Dtsch Med Wochenschr. 2011 Aug;136(33):1656-9. doi: 10.1055/s-0031-1281569. Epub 2011 Aug 10.
Five years ago a 52-year-old patient presented with arthritis of the small and large joints. Further symptoms were relapsing fever, unspecific gastrointestinal complaints with meteorism but no diarrhea, fatigue and impaired concentration. Subsequently increasing lower back pain developed. A lumbar-disc lesion was already known.
Inflammatory markers were elevated including leucocytosis. Gastroscopy with intestinal biopsies and colonoscopy remained without pathologic findings. Whipple's disease was excluded, but unspecific lymphozyte infiltration of the duodenal mucosa was described. Magnetic resconance imaging of the lumbar spine showed spondylodiscitis in L3/4 which was punctured, and polymerase chain reaction revealed Tropheryma whipplei DNA. Retrospectively, this was also found in the intestinal biopsies of three years ago.
DIAGNOSIS, TREATMENT AND COURSE: After initial exclusion of Whipple's disease an unspecific systemic inflammatory disease had been presumed, and the patient had been treated with immunomodulatory therapies in alternating combinations. Steroids improved the symptoms but an increasing dosage of steroids was required. After the detection of Tropheryma whipplei and diagnosis of Whipple's disease the patient received ceftriaxon for a period of two weeks, subsequently cotrimoxazol for one year. Inflammatory activity decreased but unspecific symptoms remained almost unaffected.
The differential diagnosis in patients with fever, elevated inflammatory markers and gastrointestinal symptoms must include Whipple's disease. A Tropheryma whipplei PCR from duodenal biopsies should be performed because of its higher sensitivity compared to histology alone.
五年前,一名52岁患者出现大小关节关节炎。进一步症状包括弛张热、伴有胃肠胀气的非特异性胃肠道不适但无腹泻、疲劳及注意力不集中。随后出现下背部疼痛加剧。腰椎间盘病变已知。
炎症标志物升高,包括白细胞增多。胃镜检查及肠道活检和结肠镜检查均未发现病理结果。排除了惠普尔病,但描述了十二指肠黏膜的非特异性淋巴细胞浸润。腰椎磁共振成像显示L3/4椎体椎间盘炎,进行了穿刺,聚合酶链反应检测到惠普尔嗜组织菌DNA。回顾性分析发现,三年前的肠道活检中也存在该菌。
诊断、治疗与病程:最初排除惠普尔病后,推测为非特异性全身性炎症性疾病,患者接受了多种免疫调节疗法交替联合治疗。类固醇改善了症状,但需要增加类固醇剂量。检测到惠普尔嗜组织菌并诊断为惠普尔病后,患者接受了两周的头孢曲松治疗,随后接受了一年的复方新诺明治疗。炎症活动度降低,但非特异性症状几乎未受影响。
对于发热、炎症标志物升高及胃肠道症状的患者,鉴别诊断必须包括惠普尔病。由于十二指肠活检的惠普尔嗜组织菌PCR检测比单纯组织学检查敏感性更高,因此应进行该项检测。