What is Whipple’s disease (WD)?

It is a chronic systemic infection by a gram positive bacterium, Tropheryma whippelli. The small intestine is most often affected, but other organs like joints, CVS and CNS may be involved too.

Discuss the epidemiology of WD?

Predominantly affects middle aged white males

Discuss the pathogenesis of WD?

Bacteria are most likely acquired by oral route. Bacteria are located in the intestinal mucosa. From here, the bacteria spread via lymphatics to mesenteric and mediastinal lymph nodes, the thoracic duct and the blood.

Discuss the clinical manifestations of WD?

• Intestines

Obstruction of mesenteric nodes leads to malabsorption with wt loss, diarrhoea and abdominal pain. Diarrhoea may be steatorrhea or watery. Occult GI bleed is common and in some cases gross GI bleeding occurs.
Abdominal and peripheral lymphadenopathy is common.

• CNS

Progressive dementia and cognitive changes, supranuclear ophthalmoplegia, altered level of consciousness and psychiatric symptoms

• CVS

Endocarditis, myocarditis and pericarditis

• Musculoskeletal

Oligo or polyarthralgias

Discuss the diagnosis of WD?

• Endoscopy- Whitish or yellow plagues are observed. Obtain 5 biopsies as distally as possible. A normal intestinal histology rules out the diagnosis
• Histology- Villi is distended by macrophage infiltrate that contain PAS positive particles and lipid droplets

Discuss the treatment of WD?
Antibiotics
Induction phase (10-14 days) – Ceftriaxone (2gm IV OD)
Maintenance phase (for at least 1 year) with an antibiotic that crosses the blood brain barrier- Trimethoprim-sulphamethoxazole