Whipple’s Disease

Question 1

What is the definition of whipple’s disease? (Intestinal lipodystrophy)

Answer 1

A rare suite is disease involving small intestine, skin, CNS, joints, heart, blood vessels, kidney, lung, Serra’s membranes, lymph nodes, spleen and liver

Question 2

What is the aetiology behind WD?

Answer 2

Infectious agents eg streptococci
Immunological factors - hummoral and cellular immunity

Question 3

What is the pathophysiology behind WD?

Answer 3

Infiltration of mesenteric lymph nodes with macrophages -> adenopathies -> perturbed (unsettled) intestinal lymph drainage

Question 4

what are the clinical features of WD?

Answer 4

Normally presents as a form of malasbsorption with diarrhoea, steatorrhea, abdominal cramps, distension, fever and marked weight loss

Question 5

What are the other symptoms and signs of WD?

Answer 5

Joint symptoms
Anaemia
Skin pigmentation
Peripheral lymphadenopathy
Neurological abnormalities
Severe malabsorption

Question 6

What is the diagnosis for WD?

Answer 6

Small intestinal mucosa is always severely involved and lesions are observed in mucosal biopsies

Lymph node or intestinal biopsy establishes diagnosis by showing foamy macrophages containing PAS positive granules

Electron microscope shows PAS positive granules to be masses of rod shaped bacilli

Question 7

What is the treatment for WD?

Answer 7

Antibiotics eg ampicillin, penicillin, tetracycline