Whipple's Disease - Symptoms, Causes, Diagnosis, and Treatment

Introduction:

Whipple's disease is a rare infection that is caused by the bacterium tropheryma whipplei belonging to the family actinomycetes commonly found in the soil. It is a systemic infection. The disease is common in farmers and those exposed to soil and animals. George Hoyt Whipple first described the disease in 1907. This disease most commonly affects the joints and the digestive system. It mainly affects the small intestine. Whipple's disease interferes with normal absorption. Whipple's disease is a multisystem disease affecting other organs, including the brain, heart, lungs, and eyes. The disease can be fatal if untreated. Whipple's disease is highly uncommon, affecting one in one million people. They are also known as intestinal lipodystrophy.

What Are the Symptoms of Whipple's Disease?

Symptoms mostly start slowly. Joint pain is a common early symptom, and gastrointestinal problems occur later. Signs of the disease vary from one person to another. Whipple's disease is not contagious.

The most common symptoms include:

• Diarrhea.

• Abdominal pain and cramps.

• Pain and stiffness in joints, particularly in ankles, knees, and fingers.

• Weight loss due to malabsorption of nutrients.

• Chronic malabsorption leads to steatorrhea (fatty foul-smelling stool).

• Bloody stools.

Other symptoms associated with Whipple's disease include:

• Inflamed joints in knees, ankles, and wrists.

• Fatigue.

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• Anemia.

Some less common signs and symptoms of Whipple's disease include:

• Fever and chills.

• Cough.

• Chest pain.

• Lymph node enlargement.

• Darkening of skin in sun-exposed areas.

• Decrease in blood protein - albumin.

• Endocarditis (infection of the heart valve).

Neurological signs and symptoms include:

• Confusion.

• Memory loss.

• Seizures.

• Poor coordination.

• Dementia.

• Difficulty in walking.

• Vision problems.

What Causes Whipple's Disease?

Whipple's disease is a bacterial infection caused by tropheryma whipplei. The bacteria first affects the mucosal lining of the small intestine, forming ulcers in the small intestine wall. The bacteria also damages villi, which are tiny finger-like projections lining the small intestine.

What Are the Risk Factors of Whipple's Disease?

Risk factors of Whipple's disease are not identified. Some factors include:

• Men of age 40-60.

• People who work outdoors and have contact with sewage and wastewater.

• Whites living in North America and Europe.

How Is It Classified?

Based on organ involvement, Whipple's disease is divided into three forms:

• Classic Whipple's disease.

• Isolated neurological Whipple's disease.

• Endocarditis associated with Whipple's disease.

How Can We Diagnose Whipple's Disease?

• Physical Examination: The doctor will check for signs and symptoms of the disease. The doctor will look for stomach tenderness, skin darkening in sun-exposed areas, enlarged lymph nodes, and heart murmur.

• Biopsy: The doctor will do an endoscopy procedure. A thin, flexible tube called an endoscope is attached with a light and a camera and is inserted through the mouth to the stomach and small intestine. The endoscope helps in viewing the inside of the stomach. The doctor takes a tissue sample (biopsy) from the small intestine lining during the procedure. This tissue sample is examined under a microscope for the presence of the disease-causing bacteria tropheryma whipplei.

• Blood Test: Complete blood count is taken to rule out anemia associated with the disease.

• Stool Test: A stool test is done if diarrhea is present.

• Polymerase Chain Reaction: It is a highly sensitive test that finds out the deoxyribonucleic acid (DNA) of the bacteria from the tissue samples. This test confirms the presence of tropheryma whipplei bacteria in the tissue.

What Is the Treatment of Whipple's Disease?

Since it is a bacterial infection, the treatment is usually done with antibiotics alone or in combination. Treatment is long-term, which takes a year, or longer but symptomatic relief is often seen in the first week. People with no brain or nervous system complications recover completely after a full course of antibiotics.

The doctor selects antibiotics that can clear infections in the small intestine lining and also cross the brain barrier to treat the bacteria that have entered the brain. Since antibiotics are used for a more extended period, antibiotic resistance should be monitored.

Initial therapy of Whipple's disease begins with two to four weeks of Ceftriaxone or Penicillin given through the vein. Following this, an oral course of Sulfamethoxazole -Trimethoprim is taken for one to two years. Alternative medications include oral Doxycycline combined with anti-malarial drug Hydroxychloroquine for one to two years.

Side effects of Ceftriaxone and Sulfamethoxazole-Trimethoprim include nausea, vomiting, diarrhea, and allergic reactions. Side effects of Doxycycline include nausea, vomiting, loss of appetite, and sensitivity to sunlight. Side effects of Hydroxychloroquine include diarrhea, stomach cramps, diarrhea, loss of appetite, and dizziness.

Some patients get relapse into the disease with worsening neurological symptoms. They are treated with antibiotics and Interferon-gamma injection, a protein that activates the immune system.

Vitamin and nutrient supplements are recommended due to malabsorption. Vitamin D, iron, calcium, folic acid, and magnesium are recommended. A diet rich in proteins and calories is recommended to compensate for the malnutrition of Whipple disease. Fluid replacement is given to prevent dehydration.

How Can We Prevent Whipple's Disease?

As of now, there is no preventive measure for Whipple's disease. Practicing good hygiene like washing hands regularly and wearing gloves while working in the soil can reduce the risk of Whipple's disease.

What Are the Complications?

• Brain damage.

• Heart valve damage.

• Inflammation of the pericardium - pericarditis.

• Inflammation of the inner lining of the heart- endocarditis.

• Weight loss.

• Nutritional deficiencies.

What Is the Prognosis?

With early diagnosis and proper treatment, the prognosis of Whipple's disease is good. Relapse is common in Whipple's disease. So, the patients should be closely monitored and undergo endoscopy with biopsy and other tests a year after the treatment. If untreated, Whipple's disease will get worse and is often fatal.

Conclusion:

Whipple's disease is a rare condition but can be life-threatening. Continuing antibiotics for up to one year can treat the disease, and relapses are common. Usually, many symptoms go away in a month once the treatment begins.

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