Abstract
BackgroundWhipple'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 presentationWe 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.ConclusionsThis report aims to raise awareness of a very rare disease that presents with a combination of symptoms mimicking other and significantly more common diseases.
Highlights
Whipple’s disease is a chronic infectious disease that primarily affects the small intestine, but several organs can simultaneously be involved
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
The first case was presented in 1907 by the pathologist George Hoyt Whipple [1] who named the disease lipodystrophy based on storage of fat in the intestinal wall and lymph nodes found at autopsy of a 36-year-old physician who died after chronic joint problems, diarrhea and weight loss [2]
Summary
Whipple’s disease is a chronic infectious disease that primarily affects the small intestine, but several organs can simultaneously be involved. 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. In 1991, Wilson et al used polymerase chain reaction (PCR) which classified the bacterium within Actinomyces [4] It was not until 2000 that isolation and cultivation of a strain of T. whipplei were reported from the heart valve of a patient with blood culture-negative endocarditis [5]. In a German study, the percentage of women was higher, 22%, with a median age of 51 at the time of diagnosis [8]
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