Rare but Not so Rare? The Evolving Spectrum of Whipple's Disease

Knowledge about Whipple's disease began to emerge in 1907, when George Hoyt Whipple recognized the first case of the disease that now bears his name. He reported the case of a 36-year-old physician with "a gradual loss of weight and strength, stools consisting chiefly of neutral fat and fa...

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Main Authors: John M Conly, B Lynn Johnston
Format: Article
Language:English
Published: Wiley 2012-01-01
Series:Canadian Journal of Infectious Diseases and Medical Microbiology
Online Access:http://dx.doi.org/10.1155/2001/547516
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author John M Conly
B Lynn Johnston
author_facet John M Conly
B Lynn Johnston
author_sort John M Conly
collection DOAJ
description Knowledge about Whipple's disease began to emerge in 1907, when George Hoyt Whipple recognized the first case of the disease that now bears his name. He reported the case of a 36-year-old physician with "a gradual loss of weight and strength, stools consisting chiefly of neutral fat and fatty acids, indefinite abdominal signs, and a peculiar multiple arthritis" (1). Findings at autopsy consisted of poly-serositis, aortic valve vegetations and deposition of fat in the intestinal mucosa and regional lymph nodes with marked infiltration by foamy macrophages (1). It was originally thought to be a disorder of fat metabolism, and the term 'intestinal lipodystrophy' was proposed. Whipple's disease has since been recognized as a rare, multivisceral, chronic disease with a clinical presentation dominated by a symptom triad of diarrhea, weight loss and malabsorption. However, digestive symptoms are often preceded for months or years by other symptoms, the most common being arthralgia, although cardiovascular, neurological or pulmonary involvement may be more prominent at times. Once considered the ideal case report, recent characterization of Tropheryma whippelii by means of broad range bacterial ribosomal DNA polymerase chain reaction (PCR) analysis (2,3) and its subsequent cultivation (4) has led to a veritable explosion of individual case reports, case series and hitherto unrecognized manifestations of the disease, such that it is now considered an underdiagnosed infectious disease (5). It is timely to provide an update on new developments in Whipple's disease.
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spelling doaj-art-be7c73a78890449492f88b631aeb42c82025-02-02T23:17:25ZengWileyCanadian Journal of Infectious Diseases and Medical Microbiology1918-14932012-01-011210.1155/2001/547516Rare but Not so Rare? The Evolving Spectrum of Whipple's DiseaseJohn M Conly0B Lynn Johnston1University Health NetworkQueen Elizabeth II Health Sciences Centre and Dalhousie UniversityKnowledge about Whipple's disease began to emerge in 1907, when George Hoyt Whipple recognized the first case of the disease that now bears his name. He reported the case of a 36-year-old physician with "a gradual loss of weight and strength, stools consisting chiefly of neutral fat and fatty acids, indefinite abdominal signs, and a peculiar multiple arthritis" (1). Findings at autopsy consisted of poly-serositis, aortic valve vegetations and deposition of fat in the intestinal mucosa and regional lymph nodes with marked infiltration by foamy macrophages (1). It was originally thought to be a disorder of fat metabolism, and the term 'intestinal lipodystrophy' was proposed. Whipple's disease has since been recognized as a rare, multivisceral, chronic disease with a clinical presentation dominated by a symptom triad of diarrhea, weight loss and malabsorption. However, digestive symptoms are often preceded for months or years by other symptoms, the most common being arthralgia, although cardiovascular, neurological or pulmonary involvement may be more prominent at times. Once considered the ideal case report, recent characterization of Tropheryma whippelii by means of broad range bacterial ribosomal DNA polymerase chain reaction (PCR) analysis (2,3) and its subsequent cultivation (4) has led to a veritable explosion of individual case reports, case series and hitherto unrecognized manifestations of the disease, such that it is now considered an underdiagnosed infectious disease (5). It is timely to provide an update on new developments in Whipple's disease.http://dx.doi.org/10.1155/2001/547516
spellingShingle John M Conly
B Lynn Johnston
Rare but Not so Rare? The Evolving Spectrum of Whipple's Disease
Canadian Journal of Infectious Diseases and Medical Microbiology
title Rare but Not so Rare? The Evolving Spectrum of Whipple's Disease
title_full Rare but Not so Rare? The Evolving Spectrum of Whipple's Disease
title_fullStr Rare but Not so Rare? The Evolving Spectrum of Whipple's Disease
title_full_unstemmed Rare but Not so Rare? The Evolving Spectrum of Whipple's Disease
title_short Rare but Not so Rare? The Evolving Spectrum of Whipple's Disease
title_sort rare but not so rare the evolving spectrum of whipple s disease
url http://dx.doi.org/10.1155/2001/547516
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