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

Abstract

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 ofTropheryma whippeliiby 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.

Whipple's Disease in an Afro-Caribbean National

“…It is caused by the organism Tropheryma whipplei, a gram positive bacillus. The incidence rate of Whipple's disease is extremely low and thus there is no accurate estimate of either the prevalence or mortality rate (6,9). The occurrence of classical Whipple's disease in Africans and persons of African descent and in the English-speaking Caribbean is rare and this is the first reported case from this region.…”

Whipple`s disease - clinical spectrum and diagnostic approach (with a contribution of eight cases)

“…One describes a reduced or even absent specific Th1-cell response along with a deficit of the macrophageal function disturbing the degradation of the bacterial antigens. That is why it is assumed that the underlying immune deficiency of the organism is responsible for WD development (3,(5)(6)(7)14). The combination of three comparatively rare diseases in the same female patient of ours, such as gluten enteropathy, WD and Crohn's disease is an interesting fact that confirms the conception of the role of the immunological factors in their pathogenesis.…”

“…Certain ocular events such as disorders of ocular muscle movements, glaucoma, keratitis, uveitis, retinitis, etc. are described, too (3,5,7,14). Heart damage presents with pericarditis, heart failure, and culture-negative endocarditis (3)(4)(5)7,12).…”

“…are described, too (3,5,7,14). Heart damage presents with pericarditis, heart failure, and culture-negative endocarditis (3)(4)(5)7,12). In some patients, there are pulmonary manifestations such as pleural effusions, interstitial lung disease, pulmonary hypertension, and lung nodules (7,12,15,16).…”

Whipple`s disease - the challenge of the clinical imitator

“…Заболяването се преименува в болест на Whipple през 1949 г. (2,4,17). Първото успешно антибиотично лечение (с хлорамфеникол) датира от 1952 г. През 1961г.…”