A disease acquired from an infectious agent called
Tropheryma whippelii, which is closely related to the actinomycetes. It results in a
malabsorption syndrome with involvement of small intestine, joints, central
nervous system and cardiovascular system. The onset of the disease can be seen at
Probably no genetic involvement.
Invasion of affected tissue by Tropheryma whippelii results in local
inflammation and organ dysfunction, as well as generalized systemic
symptoms. Appears to be associated with the human leukocyte antigen B27
Clinically evocated by arthralgias, arthritis, fever,
and diarrhea. Biopsy of duodenum or proximal jejunum reveals infiltration of
lamina propria by “foamy” macrophages with granules, which stain positively with the
periodic acid-Schiff (PAS) technique. The macrophages contain gram-positive
acid-fast negative bacilli—Tropheryma whippelii.
Can occur at any age (reports range from 3 months
to 82 years) but is more common after the fourth decade. More than 70% of
patients are male. Although the primary organ system involved is the gastrointestinal (GI)
tract, systemic symptoms and those related to involvement of other organ
systems frequently precede those attributable to the GI tract. Clinical
features can involve GI system (diarrhea, steatorrhea, gross or microscopic bleeding,
vague abdominal pain and bloating, and anorexia; these may be followed by
cachexia, fatigue, hypoalbuminemia, anemia and severe electrolyte
disturbances), articular (transient, migratory, recurring, and spontaneously
resolving arthralgias are common), neurologic (dementia, weakness, parkinsonism,
cranial nerve symptoms), and cardiac (blood culture-negative endocarditis,
constrictive pericarditis, myocarditis, conduction abnormalities). Because
of the infective nature of the disease, it is amenable to antibiotic
therapy, which is often continued for up to 36 months. Ninety percent of
patients return to normal, with a small percentage suffering relapses.
Evaluate electrolyte status,
particularly in patients with ongoing diarrhea. Other blood tests recommended include: serum
albumin, hemoglobin. Cardiac evaluation (clinical, ECG, echocardiogram).
Preoperative correction of electrolyte
abnormalities and anemia is mandatory before elective surgical procedures. Adapt anesthetic technique to cardiac involvement,
if any. Not a contagious condition.
There are no specific implications
with this condition.
Durand D, Lecomte C, Cathebras P, et al: Whipple disease: Clinical review
of 52 cases. Medicine (Baltimore) 76:3:170, 1997.
Silvestry F, Kim B, Pollack B, et al: Cardiac Whipple disease:
Identification of Whipple bacillus by electron microscopy in the myocardium
of a patient before death. Ann Intern Med 126(3):214, 1997.
Swartz MN: Whipple's disease—Past, present, and future [editorial]. N Engl J Med