Makindo Medical Notes.com

In 1907, George Hoyt Whipple described a medical missionary with a chronic illness characterized by episodes of arthritis, weight loss, cough, fever, diarrhoea, abdominal discomfort, and anaemia. Whipple disease was uniformly fatal until the 1960s when antibiotic therapy provided a response in some patients. He won a Nobel prize for this.

About

• Chronic infection with systemic disease due to Tropheryma whipplei

Aetiology

• Acid fast negative PAS positive macrophages
• Increased HLA B27

Characteristics

• Gram positive bacterium
• Culture is difficult

Clinical

• Malabsorption with steatorrhoea, bloating, flatus, cramps
• Painful joints - may be inflamed
• Pericarditis, Pleurisy and Peripheral neuropathy
• Clubbing and lymphadenopathy
• Dementia, facial myoclonus, ophthalmoplegia, encephalopathy
• Hypothalamic syndrome (hyperphagia, insomnia, polydipsia)
• Eyes - uveitis, scotoma, papilloedema

Investigations

• Anaemia (usually microcytic or normocytic), relative lymphopenia, and increased ESR
• Low Potassium, magnesium, Calcium, Albumin, Carotene, Cholesterol
• Prolonged Prothrombin time
• Raised alkaline phosphatase and Low B12, folate, ferritin
• Ix jejunal bx = small intestinal histology revealing foamy macrophages containing periodic-acid-Schiff-(PAS-) positive material. PAS-positive macrophages may infiltrate many other organs, including the heart, brain, lung, spleen, liver, and pancreas
• PCR techniques (of stools or saliva), bacteria culture, and tissue immunodetection of Tropheryma whipplei are not widely available yet.

Differential

• Coeliac disease
• Tuberculosis
• Mycobacterium avium infection of the small bowel
• Histoplasmosis
• NSAID injury to the small bowel
• non-Hodgkin lymphoma and amyloidosis

Management

• One regimen is IV ceftriaxone 2 g OD or IV Penicillin G 2–4 million every 4 hours for 2 to 4 weeks
• This is followed by PO Trimethoprim-sulfamethoxazole (960 mg twice daily) for one year
• Also treated with Doxycycline and Co amoxiclav for up to a year
• Reversible cause of dementia. Replace nutrition

References

• Whipple’s Disease: Our Own Experience and Review of the Literature