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. 2021 Mar 20;21(1):128.
doi: 10.1186/s12876-021-01664-1.

Whipple's disease: the great masquerader-a high level of suspicion is the key to diagnosis

Nikolaos Melas  1 , Rasjan Amin  2 , Paula Gyllemark  3   4 , Amil Haji Younes  1 , Sven Almer  5   6
Affiliations

Whipple's disease: the great masquerader-a high level of suspicion is the key to diagnosis

Nikolaos Melas et al. BMC Gastroenterol. .

Abstract

Background: Whipple's disease is a chronic infectious disease that primarily affects the small intestine, but several organs can simultaneously be involved. The disease is caused by a gram-positive bacterium called Tropheryma whipplei. The disease is difficult to suspect because it is rare with unspecific and long-term symptoms; it can be lethal if not properly treated.

Case presentation: We here present three patients who presented with a plethora of symptoms, mainly long-standing seronegative arthritis and gastrointestinal symptoms in the form of diarrhea with blood, weight loss, fever, and lymphadenopathy. They were after extensive investigations diagnosed with Whipple's disease, in two of them as long as 8 years after the first occurrence of joint manifestations. The diagnosis was made by PCR targeting the T. whipplei 16S rRNA gene from small bowel specimen in all three patients, and, besides from histopathologic findings from the duodenum and distal ileum in one and mesenteric lymph nodes in another patient.

Conclusions: This report aims to raise awareness of a very rare disease that presents with a combination of symptoms mimicking other and significantly more common diseases.

Keywords: Arthritis; Gastrointestinal symptoms; Tropheryma whipplei; Whipple’s disease.

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Conflict of interest statement

The authors declare no competing interests.

Figures

Fig. 1
Fig. 1
Colonoscopy. Distal ileum. Lumpy white-colored villi (see also histopathological Figs. 5 and 6) caused by ectatic lymphatic vessels that are histologically usually infiltrated by PAS-positive histiocytes. Patient 1
Fig. 2
Fig. 2
Colonoscopy. Distal ileum. See text for Fig. 1
Fig. 3
Fig. 3
Colonoscopy. Ileum. Clearly dilated lymphatic vessels in the villi (see also endoscopic Figs. 1 and 2) that make them white. Hematoxylin–eosin staining. 11x. Patient 1
Fig. 4
Fig. 4
Gastroscopy. Duodenum. Diffuse mucosal redness with white-colored villi, especially abundant on folds where there are also longitudinal fibrin and hematine-coated ulcerations. Patient 1
Fig. 5
Fig. 5
Gastroscopy. Duodenum. In lamina propria, histiocytes with PAS (periodic acid-Shiff)-positive, diastase-resistant, granulated cytoplasm is seen. 11x. Patient 1
Fig. 6
Fig. 6
Coronal CT showing enlarged lymph nodes in the small bowel mesentery (white arrows). All of them have very low attenuation suggesting fat content which is common in Whipple’s disease, Patient 3
See this image and copyright information in PMC

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