UNDERSTANDING WHIPPLE’S DISEASE: TRANSMISSION, SYMPTOMS, AND TREATMENT OPTIONS
Medically Reviewed by Dr. Sony Sherpa (MBBS)
Whipple's disease is a systemic infectious disease that affects multiple systems in the body. The cause is a bacterium called Tropheryma Whipplei, which can mimic symptoms of other conditions and make it difficult to diagnose.
This article looks at the currently known causes, symptoms, and treatment options for Whipple's disease.
What Is Whipple’s Disease?
George Hoyt Whipple first characterized Whipple's disease in 1907 as intestinal lipodystrophy, though the causative bacterium Tropheryma whipplei was identified much later in the 1990s. It is a rare condition that affects the small intestine, joints, and central nervous system.[1]
It is estimated that only 1-3 in every million people will develop Whipple's disease, making it a very rare condition. Globally, Whipple disease is rare, with most reports published in North America and Europe. The prevalence is higher in men than women and affects farmers more than other population groups.
Whipple’s Disease Causes
Whipple's disease is caused by the actinobacterium Tropheryma Whipplei[2]. This bacterium commonly resides in soil and can survive in sewage and wastewater. Experts assume that the bacteria enter the body through the mouth before reaching the small intestine, where they can cause an infection. The infection can affect multiple systems, including the immune system, small intestine, and central nervous system.
In healthy individuals, the immune system actively deals with the bacteria before symptoms of Whipple's Disease manifest. Some people may not contract any symptoms as a result.
Diseased individuals have an altered inflammatory response, impairing macrophage function and the ordinary T-cell response. The organism shares similarities with Streptococcus (groups B and G) and Shigella flexneri. Host factors, including the HLA-B27 antigen, play a pathogenic role by limiting immune detection of the organism.
The organism surrounds itself with a biofilm[3] or protein coat, which researchers theorize possibly contains the host's tissues, allowing it to invade tissues without detection. When the immune system recognizes the pathogen, it can lead to infectious relapses, a fever, and spontaneous inflammatory symptoms that may take years to materialize after the initial exposure.[4]
The bacteria can cause damage to the lining of the small intestine and disruption of the villi (microscopic hair-like projections that aid in digestion), leading to malabsorption of nutrients. The organism can spread systemically and infect multiple tissues, leading to joint and central nervous system damage and a broad spectrum of symptoms. The heart, lungs, and eyes are also known to be affected.
Risk factors for Whipple's Disease include:
- Age: Whipple's disease is more common in individuals over 55.
- Gender: Men are more likely to develop Whipple's disease than women.
- Occupation: Individuals who work in agriculture or sewage treatment may be at a higher risk of exposure to the bacteria that causes Whipple's disease.
- Weakened immune system: Immunocompromised individuals, such as those with HIV or undergoing chemotherapy, are more susceptible to Whipple's disease.
- Genetics: Whipple's Disease is associated with the HLA B27 haplotype. It may also share a connection with HLA-DRB1*13 and DQB1*06 alleles[5]. These findings suggest that those carrying these genetic alterations in immune detection make it easier to contract a T. Whipplei symptomatic infection.
Is Whipple’s Disease Contagious?
Whipple's disease is not contagious, yet there is limited evidence to suggest it is transmissible between neighbors and within families.[6]
How Is Whipple’s Disease Transmitted?
It is not fully understood how Whipple's disease is transmitted. Besides contact with contaminated soil or water, it is also possible that the bacteria may infect susceptible people through food or by inhaling contaminated dust.
Symptoms
Whipple's disease can have a widespread impact, affecting multiple systems and causing various symptoms. The symptoms may also only appear years after exposure to the organism. These factors can make diagnosing difficult, and the symptoms are often mistaken for other conditions.
The most common symptoms of Whipple's disease include:
- Weight loss
- Diarrhea
- Abdominal pain
- Joint pain and stiffness, mimicking symptoms of arthritis (also known as arthralgia)
Less prevalent manifestations comprise the following[7]:
- Fever
- Fatigue
- Anemia
- Skin hyperpigmentation
- Enlargement of various organs, such as the lymph nodes, spleen, or liver
Whipple disease neurological symptoms are not as common, yet may also include[8]:
- Dementia, including memory loss and confusion (reversible with treatment[9])
- Difficulty with coordination and balance
- Vision changes
- Headaches
- Seizures
- Muscle weakness
- Numbness or tingling in the extremities
Not all individuals with Whipple's disease experience all of these symptoms, and some are asymptomatic.
If left untreated, Whipple's disease can lead to serious complications, including malabsorption, pleural effusion (fluid buildup in the lungs), and heart problems.
Differentiating Whipple’s Disease from Other Conditions
Whipple's disease can mimic symptoms of other conditions, making it challenging to diagnose. Some conditions that present with similar symptoms to Whipple's disease include:
- Rheumatoid arthritis
- Inflammatory bowel disease[10]
- Crohn's disease
- Celiac disease
- Dementia
- Connective tissue disorders
- HIV
- Tuberculosis
- Hyperthyroidism
If you are experiencing symptoms of Whipple's disease, it is essential to seek guidance from a healthcare professional for an accurate treatment plan.
Complications of Whipple’s Disease
If left untreated, Whipple's disease can lead to severe complications, including[11]:
- Malabsorption: The damage to the small intestine caused by Whipple's disease can lead to malabsorption of nutrients, resulting in malnutrition and weight loss.
- Pleural Effusion: The organism can cause fluid to build up between the pleura that externally lines the lungs, leading to chest pain and respiratory problems[12].
- Heart Problems: If the organism reaches the heart, it can injure the heart valves, leading to a heart murmur, endocarditis, and other heart problems. In extreme cases, a lack of treatment can lead to heart failure.[13] Heart valve tissue was one of the first occasions T. Whipplei was successfully cultured in a laboratory.
Recurrence after treatment and brain damage are other possible complications.
How to Diagnose Whipple's Disease
Diagnosing Whipple's disease is problematic as the symptoms often mimic those of other conditions and tend to appear years after exposure.
Your doctor will conclude a physical exam and order several tests to confirm the diagnosis.[14] These may include:
- Blood tests to inspect for anemia and signs of infection.
- A biopsy of the small intestine is done to assess for any damage and the presence of bacteria. During this procedure, the doctor will use a non-invasive endoscope, an adjustable tube with a camera at the end, and surgical tools.
- Imaging tests, often X-rays or MRI scans, to rule out other conditions, such as arthritis.
Treatment Options for Whipple’s Disease
The primary treatment for Whipple's disease is long-term antibiotic therapy. The most commonly used antibiotic is Trimethoprim Sulfamethoxazole[15], taken for 1-2 years. In some cases, Ceftriaxone or doxycycline can treat recurrence or treatment-resistant cases.
Antibiotic treatment can lead to Immune Reconstitution Inflammatory Syndrome (IRIS). In IRIS, the immune system starts to recover and may overreact, leading to various inflammatory symptoms, including gastrointestinal symptoms, visual disturbances, fever, and arthritis.
In addition to antibiotics, treatment for Whipple's disease may also include fluid replacement to address malabsorption and vitamin and nutrient supplements to address any deficiencies.[16]
Diet for Whipple’s Disease
Individuals with Whipple's disease may need to follow a specific diet to address malabsorption and nutrient deficiencies. An appropriate diet may include a high protein and calorie content and a low fat and fiber content.[17]
A registered dietitian with relevant experience can assist you with creating a diet plan that meets your needs.
How Long Can You Have Whipple’s Disease?
With proper treatment, most individuals with Whipple's disease can make a full recovery. However, it is essential to continue taking antibiotics for the recommended duration to prevent a relapse.
In some cases, symptoms relapse after treatment, and long-term follow-up care may be necessary.
Prognosis and Management of Whipple’s Disease
With early diagnosis and proper treatment, the prognosis for Whipple's disease is generally reasonable. The patient starts to recover within 2-3 weeks. However, if left untreated, it could have serious consequences and can be fatal.
Following your doctor's treatment recommendations and attending regular follow-up consultations to observe your progress are crucial.
How to Prevent Whipple’s Disease
As the exact mode of transmission for Whipple's disease is not fully understood, there are no specific measures to prevent the condition. However, you can lessen your risk by evading contact with contaminated soil or water and ensuring good hygiene, especially regular hand washing.
Current Medical Research on Whipple’s Disease
Research is ongoing to understand Whipple's disease better and develop more effective treatments. Current research areas include:
- Studying the genetic link to Whipple's disease and identifying potential risk factors.
- Investigating the immune system's role in the development and progression of Whipple's disease.
- Inventing new diagnostic equipment and treatments for Whipple's disease.
Conclusion
Whipple's disease is rare and can significantly impact the body and its systems. Seeking medical attention if you are experiencing symptoms of the condition is necessary to receive an accurate diagnosis and begin treatment. With proper care, most individuals with Whipple's disease can make a full recovery and manage their symptoms effectively.
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