Whipple disease affects the digestive system that restricts the absorption of nutrients. Treatment of Whipple disease begins with antibiotics to stop the bacterial activity.

Whipple’s Disease

Whipple’s  Disease

Online Consultation

Iqra Mazhar

Overview

Whipple&#39;s disease is essentially a chronic infectious disease which affects your small intestine, but can also affect multiple organs all at once. The infection is caused by a specific gram-positive bacteria called Tropheryma whipplei. It is often a challenge to diagnose the illness as it is very rare with nonspecific and chronic symptoms. If not properly treated, Whipple&rsquo;s disease can turn fatal.

Whipple disease is a systemic disorder that not only affects the gastrointestinal tract, but also other systems like the cardiovascular, central nervous system and vascular systems. Early diagnosis can help decrease the morbidity and mortality rate of Whipple disease. 
&nbsp;

Whipple disease is a rare disorder worldwide, with Europe and North America reporting the most number of cases. Statistics show that the overall incidence of Whipple disease is around one to three in every one million people while the mean age of 55 years<a href="#accordion">[8]</a>. Moreover, the disease is much more common in males compared to females, with a ratio of 4:1<a href="#accordion">[8]</a>.

The disorder is associated with the HLA-B27 haplotype which puts one at a greater risk of certain autoimmune disorders. An autoimmune disorder refers to a condition in which your immune system mistakenly attacks and destroys healthy body tissue<a href="#accordion">[1]</a>.

A population-based study conducted in the United States found a total of 35,838,070 individuals in an active disease state between November 2012 and November 2017<a href="#accordion">[2]</a>. Data were derived from 26 major healthcare systems in the United States. Approximately 350 of these individuals had a CT-supported diagnosis of Whipple&rsquo;s disease<a href="#accordion">[2]</a>. Moreover, the overall prevalence of Whipple&rsquo;s disease was reported as 9.8 cases per 1 million<a href="#accordion">[2]</a>. Gender did not influence the overall prevalence. However, on the basis of ethnicity, the prevalence of Whipple&rsquo;s disease was higher in Caucasians, non-Hispanics, and individuals who were older than 65 years of age<a href="#accordion">[2]</a>.

Furthermore, patients diagnosed with Whipple&rsquo;s disease showed a greater association with signs and symptoms of <a href="https://merisehat.pk/disease/arthritis">arthritis</a>, neurological diseases, endocarditis, <a href="https://merisehat.pk/disease/diabetes-type-1-and-2">diabetes</a>, malignancy, <a href="https://merisehat.pk/disease/dementia">dementia</a>, <a href="https://merisehat.pk/disease/vitamin-d">vitamin D </a>deficiency, iron deficiency, chemotherapy, weight loss, abdominal pain, and lymphadenopathy. 
&nbsp;

Whipple&rsquo;s disease has no types. 
&nbsp;

Signs and Symptoms

Whipple&rsquo;s disease mainly affects your digestive system, majorly, your small intestine. However, it may also affect your bones, joints, other organs, and the nervous system.

The most common symptoms of Whipple&rsquo;s disease include<a href="#accordion">[4]</a>:

<ul>
	<li>Abdominal pain</li>
	<li><a href="https://merisehat.pk/disease/diarrhea">Diarrhea</a></li>
	<li>Joint pain</li>
	<li>Unexplained weight loss 
	&nbsp;</li>
</ul>

Around 20% to 40% of people who are infected with Tropheryma whipplei, also develop neurological problems<a href="#accordion">[4]</a>. Upon involvement of the nervous system, one may experience:

<ul>
	<li>Loss of balance</li>
	<li>Difficulty swallowing, (dysphagia)</li>
	<li>Memory loss.</li>
	<li>Confusion.</li>
	<li>Personality changes</li>
	<li><a href="https://merisehat.pk/disease/absence-seizures">Seizures</a></li>
	<li>Speech problems</li>
	<li>Uncontrollable eye or facial movements 
	&nbsp;</li>
</ul>

Less common signs of Whipple&rsquo;s disease include:

<ul>
	<li><a href="https://merisehat.pk/disease/anemia">Anemia </a>(low red blood cells)</li>
	<li>Cough</li>
	<li>Dark skin patches</li>
	<li>Fatigue</li>
	<li>Fever</li>
	<li>Joint pain</li>
	<li>Swollen lymph nodes 
	&nbsp;</li>
</ul>

Causes

Whipple disease is caused by a type of bacterium called Tropheryma whipplei. It is a soil-dwelling organism, which explains the high disease prevalence among farmers.

When the bacteria invade your body, they quickly spread throughout the body, affecting the intestinal epithelium, macrophages, capillary and lymphatic endothelium, liver, brain, heart, lung, kidneys, bone marrow, and skin. All of these sites lack an inflammatory response to Tropheryma whipplei. Moreover, since the organism exerts no cytotoxic effects upon the host cells, it leads to an accumulation of Tropheryma whipplei at the sites of infection.

The organism initially affects the lining of your small intestine&rsquo;s mucosa and leads to the formation of small sores (lesions) within the walls of the intestine. The bacteria are also responsible for damaging the hair-like projections (villi) lining the small intestine.

Not much information is available on the organism, however, it is present readily in the environment. Scientists are unsure about its origin and how it spreads in humans. There is a chance that you may carry the bacteria, but never develop the disease. Some researchers even believe that patients with the disease may have an exclusive genetic deformity in their immune system response that makes them susceptible to diseases, and because of the lost efficiency of the immune system, get exposed to the bacteria<a href="#accordion">[3]</a>.

&nbsp;

Although there are no specific tests to screen a person for Whipple&rsquo;s disease, a detailed history of the parents along with the relevant signs and symptoms presented by the patient can warrant diagnostic tests to be carried out. 
&nbsp;

Diagnosis

Diagnosing Whipple&rsquo;s disease is fairly complicated, because the symptoms are quite similar to other diseases which range from celiac disease to neurological disorders. It is important to discuss the possibility of having Whipple&rsquo;s disease, especially if the current management plan is not helping you as much as it should. The risk of it being misdiagnosed can be fatal. Hence, the following is a list of differential diagnoses that may mask disease: 
&nbsp;

1.&nbsp;Malabsorptive Diseases

These are diseases with small intestinal involvement for example coeliac disease, <a href="https://merisehat.pk/disease/tuberculosis">tuberculosis</a>, Mycobacterium Avium infection of the small intestine, histoplasmosis, and non-steroidal anti-inflammatory drug-induced injury to the small bowel 
&nbsp;

2.&nbsp;Infiltrative Diseases

These include<a href="https://merisehat.pk/disease/non-hodgkins-lymphoma"> non-Hodgkin lymphoma</a> and <a href="https://merisehat.pk/disease/amyloidosis-24594">amyloidosis</a>. 
&nbsp;

3.&nbsp;Coexistence Of Conditions

If <a href="https://merisehat.pk/disease/diarrhea">diarrhea</a>, malabsorption, weight loss, seronegative arthritis, lymphadenopathy, and low-grade fever specifically exist with the typical symptoms, it should raise suspicion for the possible diagnosis of Whipple&#39;s disease. Thus, a biopsy of the intestinal mucosa must be conducted. Moreover, other causes of malnutrition (such as kwashiorkor) must be excluded. 
&nbsp;

4. Connective Tissue Disease

If there are signs of pulmonary hypertension, primary pulmonary hypertension should first be considered. Occasionally, it is extremely important to exclude inflammatory bowel disease with enteropathy-associated arthritis (joint pain due to intestinal disease), <a href="https://merisehat.pk/disease/hyperthyroidism">hyperthyroidism</a>, and <a href="https://merisehat.pk/disease/aids">AIDS</a>. 
&nbsp;

5. <a href="https://merisehat.pk/disease/addisons-disease">Addison&#39;s Disease</a>

Primary adrenocortical insufficiency must be ruled out if the patient is complaining of <a href="https://merisehat.pk/disease/orthostatic-hypotension">orthostatic hypotension</a> (low blood pressure that happens when standing after sitting or lying down), skin hyperpigmentation (darkening of skin tone), low serum sodium, (hyponatremia), and high serum potassium (hyperkalemia). 
&nbsp;

<ul>
	<li>Once your healthcare practitioner is fairly certain that it is Whipple&rsquo;s disease, further diagnostic procedures are advised which may include<a href="#accordion">[6]</a>:</li>
</ul>

1.&nbsp;Endoscopy

The first and most crucially alarming sign your doctor will observe is intestinal lesions. An endoscopy involves the insertion of a small flexible tube all the way down your throat to the small intestine. The tube has a small camera attached to it and enables your doctor to look at the condition of your intestinal walls. If the results show thickened walls with creamy, ragged covers, it is a potential sign that you have Whipple&rsquo;s. 
&nbsp;

2.&nbsp;Biopsy

During the same endoscopic procedure, your doctor may extract a tissue sample from your intestinal walls to test for the presence of the T. whipplei bacteria. The process is referred to as a biopsy and confirms the presence of the infection. 
&nbsp;

3.&nbsp;Polymerase Chain Reaction (PCR)

This is an extremely sensitive test that multiplies the amount of the DNA of T. whipplei from your tissue samples to confirm its presence.

4.&nbsp;Blood Tests

Your doctor might order a complete blood count. This will help determine if you have a low count of red blood cells and albumin, which are both signs of anemia (a sign of Whipple&rsquo;s disease). 
&nbsp;

Treatment

Getting rid of the bacteria can be hard and the treatment uses a multidisciplinary approach. Whipple&rsquo;s disease treatment includes<a href="#accordion">[4]</a>: 
&nbsp;

<ul>
	<li>
	<h5>Antibiotics</h5>
	</li>
</ul>

You may initially be started with intravenous (IV) antibiotics. A variety of drugs may also be administered orally for up to one year. Some patients can take oral antibiotics in combination with hydroxychloroquine (an anti-malarial drug). 
&nbsp;

<ul>
	<li>
	<h5>Fluid Replacement</h5>
	</li>
</ul>

To avoid dehydration, you might be started on IV solutions. If not through the IV route, you may drink electrolyte beverages in order to make up for the lost fluids and salt. 
&nbsp;

<ul>
	<li>
	<h5>Vitamins And Nutrients</h5>
	</li>
</ul>

Your doctor can even recommend taking additional supplements so that you have the vitamins and nutrients readily available as your intestines are unable to absorb them efficiently. 
&nbsp;

If Whipple&rsquo;s disease is left untreated, it can aggravate and even prove to be fatal. The process behind is simple - malabsorption. It takes place when your body is not efficient enough to process fat or any other nutrients for that matter. Consequently, your body lacks essential nutrients and vitamins.

Whipple&rsquo;s disease can cause the fluids in your body to build up between the thin membranes called pleura, which essentially line the outer surface of your lungs, giving rise to a condition called pleural effusion (PE). PE causing chest pain and shortness of breath and may require draining of the fluid.

A heart murmur can also occur. 
&nbsp;

There is not enough information available about the organism that causes Whipple disease, therefore, the risk factors of the disease haven&#39;t been clearly studied. However, from the limited information&nbsp; available, the following factors are most likely to increase one&rsquo;s risk of Whipple&rsquo;s disease: 
&nbsp;

<ol>
	<li>Men aged between 40 to 60</li>
	<li>Caucassian ethnicity (North America and Europe)</li>
	<li>Farmers or people who work outdoors</li>
	<li>People who work with sewage or an in constant contact with it 
	&nbsp;</li>
</ol>

There are no definite preventive measures that can help you prevent Whipple&rsquo;s disease, however, some basic measures can be taken, like<a href="#accordion">[4]</a>: 
&nbsp;

1. Wear gloves while you are working outdoors, especially if it involves soil and water sources.

2. Wear a mask for your work i.e. sewage workers.

3. Wash your hands thoroughly after working in dirt, mud or contaminated water. 
&nbsp;

Whipple&rsquo;s Disease commonly presents with arthralgia (joint pain) and/or arthropathy (disease of the joints). These manifestations often occur years before the diagnosis, causing doctors to prescribe tumour necrosis factor inhibitors (TNFI) for joint troubles. However, if given in a misdiagnosed case, these therapies have been reported to aggravate a subclinical type of Whipple&rsquo;s disease. Researchers studying samples taken during a duodenal biopsy found increased expression and circulating levels of IFN-&gamma; (a cytokine that enhances antigen expression for your immunity to target it) in patients diagnosed with Whipple&rsquo;s disease while under TNFI therapy. Thus, demonstrating that TNFI aggravates dormant or subclinical Whipple&rsquo;s disease<a href="#accordion">[7]</a>. 
&nbsp;

Key Points

<ul>
	<li>Whipple&#39;s disease is essentially a rare, chronic infectious disease which affects your small intestine</li>
	<li>It is caused by a specific gram-positive bacteria called Tropheryma whipplei</li>
	<li>If not properly treated, Whipple&rsquo;s disease can turn fatal. Therefore, early diagnosis can help decrease morbidity and mortality</li>
	<li>The disorder is associated with the HLA-B27 haplotype which puts one at a greater risk for developing certain autoimmune disorders</li>
	<li>Statistics show that the overall incidence of Whipple disease is around one to three in every one million people, while the mean age is 55 years.</li>
	<li>Whipple&rsquo;s Disease is much more common in males, compared to females, with a ratio of 4:1</li>
	<li>The most common Whipple&rsquo;s disease symptoms include abdominal pain, diarrhoea, joint pain, and unexplained weight loss</li>
	<li>Around 20% to 40% of people who are infected with Tropheryma whipplei, also develop neurological problems</li>
	<li>Lesser common signs of Whipple&rsquo;s disease include anaemia (low red blood cells), cough, dark skin patches, fatigue, and fever</li>
	<li>There can be a chance that you carry the bacteria but never develop the disease</li>
	<li>Although there are no specific tests to screen a person for Whipple&rsquo;s disease, a detailed history of the parents along with the relevant signs and symptoms presented by the patient can warrant diagnostic tests to be carried out</li>
	<li>It is important to discuss the possibility of you having Whipple&rsquo;s Disease, especially if the current management plan is not helping you as much as it should</li>
	<li>Differential diagnoses that may mask the disease include malabsorptive diseases, infiltrative diseases, the coexistence of chronic conditions and connective tissue disease</li>
	<li>In Whipple&rsquo;s, endoscopy may show thickened walls with creamy, ragged covers</li>
	<li>During the same endoscopic procedure, your doctor may extract a tissue sample (biopsy) from your intestinal walls to test for the presence of the T. whipplei bacteria</li>
	<li>Polymerase chain reaction (PCR) is an extremely sensitive test that increases the amount of the DNA of T. whipplei from your tissue samples to confirm its presence</li>
	<li>Your doctor might order a complete blood count. This will help determine if you have a low count of red blood cells and albumin, which are both signs of anaemia ( an indication of Whipple&rsquo;s disease)</li>
	<li>Whipple&rsquo;s Disease treatment includes antibiotics, IV fluids, and supplements</li>
	<li>Malabsorption takes place when your body is not efficient enough to process fat or any other nutrients for that matter. Thus, your body begins to lack essential nutrients and vitamins</li>
	<li>Whipple&rsquo;s Disease can cause the fluids in your body to build up between the thin membranes called pleura, which essentially line the outer surface of your lungs, giving rise to a condition called pleural effusion (PE)</li>
	<li>Men aged between 40 to 60 years of age, and farmers or people who work outdoors and are in constant contact with sewage are at a higher risk of developing Whipple&rsquo;s disease</li>
	<li>It can be prevented by maintaining good hygiene, and wearing essential gear while working with soil or in the sewers. 
	&nbsp;</li>
</ul>

[1] Bureš, J., Kopáčová, M., Douda, T., Bártová, J., Tomš, J., Rejchrt, S., & Tachecí, I. (2013). Whipple's Disease: Our Own Experience and Review of the Literature. Gastroenterology research and practice, 2013, 478349.

[2] Elchert, J. A., Mansoor, E., Abou-Saleh, M., & Cooper, G. S. (2019). Epidemiology of Whipple's Disease in the USA Between 2012 and 2017: A Population-Based National Study. Digestive diseases and sciences, 64(5), 1305–1311.

[3] Whipple's disease. Mayo Clinic. Published November 8, 2022. Accessed November 24, 2022.

[4] Whipple's disease: What is it, causes, symptoms & treatment. Cleveland Clinic.

[5] Whipple's disease. Mayo Clinic. Published November 8, 2022. Accessed November 24, 2022.

[6] Johnson S. Whipple's disease: Symptoms, causes, and treatments. Healthline.

[7] Boumaza A, Mezouar S, Bardou M, Raoult D, Mège J-L, Desnues B. Tumor necrosis factor inhibitors exacerbate Whipple’s disease by reprogramming macrophage and inducing apoptosis.

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