Whipple disease

Description

Rare multi-system disorder secondary to chronic bacterial infection. Affecting the gastrointestinal tract most frequently. Chronic infection of the intestinal mucosa with the bacterium Tropheryma whipplei, leads to a lymphostasis; abdominal pain; malabsorption syndrome with diarrhoea; and weight loss.

History

1907 – George Hoyt Whipple described in meticulous detail the fatal course in a young physician of a previously unrecognised ‘intestinal lipodystrophy’

Whipple named the disorder “intestinal lipodystrophy” based on his belief that altered fat metabolism played a role in its pathogenesis. He also noted numerous rod-shaped organisms in a silver stained lymph node from his case, but the significance of this observation was not apparent in 1907.

1952 – The infectious nature of Whipple disease became apparent when antibiotics trials demonstrated that patients could be cured of this usually fatal disease.

Later, electron microscopy revealed the presence of unique bacillary structures within tissues from patients with Whipple disease. Clinical response to antibiotics was associated with disappearance of bacilli. Reappearance of bacilli heralded clinical relapse.

Despite the accumulated evidence of a bacterial etiology for Whipple disease, initially no organism was reproducibly cultured from tissues of affected patients.

Associated Persons

• George Hoyt Whipple (1878-1976)

Alternative names

• Whipple’s disease
• Intestinal lipodystrophy

References

• Whipple GH. A hitherto undescribed disease characterized anatomically by deposits of fat and fatty acid in the intestinal and mesenteric lymphatic tissues. Bulletin of the Johns Hopkins Hospital. 1907;18:382–393.
• Paulley JW. A case of Whipple’s disease (intestinal lipodystrophy). Gastroenterology. 1952 Sep;22(1):128-133. [PMID 12980233]
• Haubrich WS. Whipple of Whipple’s disease. Gastroenterology. 1999;117(3):576