Table of contents
What is Whipple’s disease?
Whipple’s disease is an infectious disease caused by Tropheryma whipplei- a bacterium and a causative organism. It is a rare systemic disease affecting some or even whole body parts. It mainly affects the digestive system and decreases the body’s absorption of nutrients. Also, changes in the joints, lymph nodes, heart, and lungs are seen.
Whipple’s disease is caused by a bacterium called Tropheryma whippeli. This bacillus-like bacterium is widespread in the environment but is found primarily in water treatment plants. The conditions that contribute to the infection are not yet known.
However, several studies indicated that immunogenetics is a possible factor for etiopathogenesis. Defective functions of HLA genes and a decrease in the immune response of T-lymphocytes (particularly in Th1 cells) show a high risk of developing Whipple’s disease. But so far, these studies have not been confirmed.
The symptoms of Whipple’s disease differ depending on which organs are most affected; and the stage of the disease.
The most common symptom is intestinal malabsorption. It causes:
- Weight loss
- Abdominal pain
Other symptoms include:
- Polyarthralgia (joint pain)
- Arthritis (joint inflammation)
- Changes in skin pigmentation
- Chronic cough and pleural pain
Less common symptoms of the disease include:
- Peripheral edema
- Lymphadenopathy (swollen lymph nodes, especially mesenteric)
- Inflammation of the eyes
- Recurrent tenosynovitis
Symptoms by stage
At stage 1 of the disease, the patient complains of intermittent symptoms like:
- Joint pain – can occur for a short time; sometimes arthritis is diagnosed, but it appears rapidly and suddenly disappears.
- Periodic increase in body temperature, which cannot be associated with colds or inflammatory diseases
- Peripheral lymph nodes increase in size but, at the same time, are painless.
- Hyperpigmentation- mainly on the face and thighs
Stage 1 Whipple’s disease can last several years, up to a decade. Doctors prescribe treatment based on the patient’s complaints and temporarily relieve the patient, but the infection continues to develop.
At the onset of stage 2, the disease affects the digestive system. A patient may experience:
- Frequent diarrhea – up to 15 times a day, regardless of the quantity and quality of food taken.
- Abdominal pain immediately after a meal
- A sharply decreasing body weight- up to cachexia.
At stage 3, Whipple’s disease is specifically systemic – not only the intestines but other organs/systems are affected. It is during this period that pronounced symptoms appear:
- Cough, shortness of breath, separation of a large amount of sputum, and in some cases, bronchospasm appears
- Signs of endocarditis – changes in the heart wall;
- “Night blindness” and inflammation of the eyes (mainly the organ membrane is affected).
Additionally, it also affects the nervous system. Whipple’s neurological symptoms during stage 3 include dementia, memory loss, lack of attention, difficulty walking, convulsions, and paralysis. All these symptoms do not appear instantly; at first, there is frequent irritation, depression, and migraine.
Convulsions first appear with slight twitching of the upper/lower extremities, and paralysis begins to affect the patient gradually (usually from the upper extremities)
How is Whipple’s disease diagnosed?
The diagnosis of Whipple’s disease is difficult since most of the signs and symptoms are similar to other conditions. The doctor will advise the patient to undergo a couple of tests to confirm the diagnosis. They are listed below:
- Physical examination- During the physical examination, the doctor will examine the patient thoroughly. The doctor will look for any signs and symptoms that suggest the presence of this condition.
- Endoscopy– During this procedure, a small flexible tube and camera are placed inside the mouth and slowly passed into the esophagus, stomach and intestine. The doctor will observe the walls of the intestine. Thick walls with creamy ragged covers are potential signs of Whipple’s disease.
- Biopsy– During this procedure, a small sample of abnormal tissue is taken from the intestinal walls and sent for pathological examination to confirm the disease. This procedure is carried out during endoscopy.
- Polymerase chain reaction- This is considered a highly sensitive test that amplifies the DNA of the T. whipplei from the tissue sample.
- Blood tests- They include: Complete blood count, serum albumin, prothrombin tests, and serum carotene. These tests can detect certain conditions associated with Whipple’s disease.
- Imaging studies- They include CT scans of the abdomen and MRI scans. These tests may suggest the presence of malabsorption.
The doctor also performs a differential diagnosis for Whipple disease for conditions that cause similar symptoms, including:
- Seronegative polyarthritis
- Ankylosing spondylitis;
- Culture-negative endocarditis
- Malabsorption syndrome
- Cerebrovascular diseases
- HIV infection
- Atypical mycobacteriosis
How is Whipple’s disease treated?
Treatment for Whipple’s disease involves using antibiotics to kill bacteria intravenously and then oral therapy for 12 months. The most commonly used drugs are tetracycline, chloramphenicol, chlortetracycline, sulfasalazine, ampicillin, penicillin, and trimethoprim/sulfamethoxazole. Currently, the most common therapy for Whipple’s disease is the combination of trimethoprim + sulfamethoxazole. Another alternate combination of drugs consists of cephalosporins or penicillins followed by tetracycline. The treatment is long-term and can last for at least one year. In most cases, a complex conservative technique is used, including dietary nutrition and medication. Antibiotic therapy usually correlates with good results.
Fluid replacement- The doctor advises intravenous fluids to the patients to prevent dehydration.
Vitamins and nutrients- The doctor advises the patient to take vitamins and nutrient supplements that the intestine can’t absorb.
Iron supplements- It is advised in patients who are anemic.
Corticosteroids and NSAIDs- Corticosteroids are advised to ease inflammation, while NSAIDs such as ibuprofen are advised.
Diet during treatment
During the treatment regimen, it is necessary to eat healthily and keep a balanced diet so that the body can recover from nutritional deficiencies. Professionals like nutritionists may select the diet plan depending on the conditions. It is necessary to consistently maintain immunity, so infections and bacteria do not spread to other systems or organs outside the intestines. Drug therapy, along with the intake of vitamins B12, A, E, K, and D, as well as folic acid and iron, is complimentary. Diet is a component of treatment. Common foods recommended during treatment:
- Sour milk products such as yogurt or kefir (lactic acid)
- Cooked or baked seasonal vegetables (carrots, potatoes)
- Boiled eggs or omelet
- Dairy-free cereals (rice, semolina, buckwheat)
- Vegetable soups in low-fat broth
- Low-fat dietary meats and fish in baked, stewed, or boiled form
Foods to avoid:
- Smoked meats
- Raw vegetables
- Spicy, fatty, fried, and acidic foods
- Alcohol and black coffee
- Fast-food products
a) Whipple’s disease is a rare systemic disease caused by a bacterium called Tropheryma whipplei. The treatment course is long-term. This condition causes symptoms like lymph nodes, malabsorption, diarrhea, abdominal pain, and weight loss.
b) The disease got its name from the name of the American pathologist George Whipple, who first discovered it and described its characteristic symptoms in 1907.
Whipple’s disease is a disease in which nearly all organs can be affected by the bacterium Tropheryma whipplei. It mainly affects the small intestine but can cause complications to other organs, including the spleen, heart, lungs, liver, kidneys, joints, eyes, and the central nervous system.
The disease may lead to complications like:
a) Lack of adrenal function
c) Cerebral edema
d) Extreme exhaustion of the body.
e) Pulmonary, renal, and heart failure
Some complications of Whipple’s disease are treated with surgery. For example, for hydrocephalus, bypass surgery is performed. To avoid complications, the patient needs to be attentive to their own body and should consult a doctor on time. There is no prophylaxis for Whipple’s disease, but it is rare: one case occurs in more than 1 million of the world’s population. To avoid it, the patient has to strengthen their immune system, lead a healthy lifestyle, and give the body time to rest and recover.
1) The disease is common in people working at wastewater treatment plants and in farmers who are in contact with soil and animals, suggesting that the infection is from these sources.
2) Men: Whipple’s disease mainly affects men between the ages of 30-60.
3) White people in North America and Europe
Symptoms such as weight loss, diarrhea, weakness, and swollen lymph nodes necessitate the differential diagnosis of Whipple’s disease with the following:
2) Cancerous tumor
3) Crohn’s disease
4) Ulcerative colitis
5) Zollinger-Ellison syndrome
8) Addison’s disease
Whipple’s disease is considered to be an extremely rare condition. Approximately 10 out of every 1 million population are diagnosed with Whipple’s disease annually in the USA.
No, Whipple’s disease is not contagious like cold or flu. The disease can’t spread by being near the infected person.
The person has to follow the rules below to prevent getting infected with Whipple’s disease. They are:
a) Wearing gloves while working in outdoor soil and water sources.
b) Sewage workers or someone working near the contaminated area should wear masks properly.
c) Wash hands thoroughly after working in dirt, mud or dirty water area.
Most of the patients who are diagnosed and treated early have a good prognosis. Patients who are left untreated have a bad prognosis. Clinical remission occurs in about 70% of the patients.
Whipple’s disease is a progressive and potentially fatal condition if left untreated. Although the condition is rare, associated death is continued to be reported.
Some researchers believe that people diagnosed with Whipple’s disease may have a genetic defect in their immune system responses, making them more likely to become sick when exposed to the bacteria.