Whipple disease: symptoms | treatment | histology

Whipple disease: symptoms, treatment, and histology 

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Whipple disease definition

Whipple disease is a systemic disease that is caused by Tropheryma whippelii, a gram-positive bacteria, first described in 1907 by George Hoyt Whipple.

The disease also tends to affect the joints, cardiovascular system and the central nervous system, although its common manifestation is a malabsorption syndrome because the mucosal lining of the small intestine is targeted and small lesions within the intestinal wall form.

The bacteria also damage the small intestine's fine, hairlike projections (villi). It may occur at any age, but in the fourth to sixth decades, it most frequently affects white males.

Whipple disease symptoms

Whipple disease has a lot of signs and symptoms that make it easy to diagnose. In Whipple disease, gastrointestinal signs and symptoms are common and may include.

• Cramping and abdominal pain that can escalate after meals

• Diarrhoea, steatorrhea (an excessive amount of fat in the stool)

• Weight loss, linked with nutrients malabsorption Anemia, with weakness and fatigue due to inadequate absorption of iron, folic acid, and vitamin B12

• Oedema (fluid retention in the body's tissues) due to decreased protein absorption

Other common symptoms and signs associated with Whipple's disease include:

• Inflamed joints, especially the wrists, ankles and knees

• Weakness

• Fever

• Cough

• Chest pain

• Enlarged lymph nodes

• Difficulty in walking

• Memory loss

• Confusion

• Skin darkening (hyperpigmentation)

Whipple disease histology

In most cases, the diagnosis of Whipple's disease is confirmed by histological endoscopic biopsy of the duodenum showing invasion of lamina propria with PAS-positive macrophages containing gram-positive bacilli and lacteal dilatation.

While the PAS stain is less sensitive and common to extra-intestinal Whipple's disease, the diagnosis is confirmed by PCR.

Because asymptomatic central nervous system infection occurs in 45% of patients, the examination of the cerebrospinal fluid by PCR for T whippelii be performed routinely. The specificity of PCR is 100% and the sensitivity of PCR is 95%.

Whipple disease differential diagnosis

We easily distinguish Whipple's disease from other mucosal malabsorptive diseases, such as celiac sprue, by small bowel biopsy.

Whipple disease should be considered in patients presenting with symptoms of malabsorption, fever of unknown origins, lymphadenopathy, arthritis, seronegative, culture-negative endocarditis or multisystemic disorder.

Whipple disease complications

Sometimes there can be very severe complications of Whipple disease which include,

• Nutritional deficiencies

• Unintentional weight loss

• Brain damage

• Heart valve damage

• Skin damage

• Arthritis

Whipple's disease laboratory testing

Patients may have laboratory abnormalities if severe malabsorption is present. It is possible to recommend the diagnosis on the basis of the signs and symptoms, the major ones include.

Diarrhoea, steatorrhea, stomach pain, loss of weight, fever, and neurological signs of migratory arthropathy.

• Steatorrhea (light, bulky stools) increased fecal fat, decreased serum cholesterol, decreased serum carotene, vitamin A, and vitamin D in laboratory results. The malabsorbed nutrients are Triglycerides, fatty acids, phospholipids, cholesterol. Fat-soluble vitamins; A, D, E, K.

• Lab findings in diarrhoea increased weight and stool volume, increased stool osmolality gap and increased fecal fat, same malabsorbed nutrients are fats and carbohydrates.

• In milk intolerance (bloating, cramps, diarrhoea) lab finding is an abnormal lactose tolerance test and as we know the malabsorbed nutrients is lactose.

Blood tests: Most conditions associated with Whipple's disease, particularly anaemia and low albumin concentrations, can be diagnosed by blood checks such as full blood counts.

Biopsy: The intestinal tissue is microscopically analyzed for the presence of disease-causing bacteria and their lesions, and specifically for Tropheryma whippelii bacteria, which reveals PAS-positive macrophages in the lamina propria containing non-acid- fast gram-positive bacilli.

Whipple disease diet

A Whipple disease and malabsorption patient may need;

• Vitamins

• Nutritional supplements

• A diet rich in protein and calories

Whipple disease treatment

Antibiotic treatment, including in some cases with neurological intervention, results in significant clinical progress over a few weeks.

There is no known optimum regimen. Usually, the full therapeutic response is apparent within 1-3 months; however, up to one-third of patients may report relapse after discontinuation of the medication.

Therefore, sustained treatment is needed for at least 1 year. Medicines that cross the blood-brain barrier are preferred. Therapy for Whipple's disease continues of IV (intravenous) ceftriaxone or penicillin for 2 to 4 weeks.

Oral treatment of SMZ-TMP or sulfamethoxazole-trimethoprim for 1 to 2 years will be recommended after this initial treatment. A shorter time of therapy of antibiotics may lead to a relapse.

If the Whipple disease is left untreated, it is ultimately fatal. The aim of treatment is to avoid this progression since certain neurological symptoms can be permanent. After treatment, patients should be followed closely for signs of recurrence of the disease.

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