Whipple Disease

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 all ages.

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 (HLA-B27) haplotype.

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.