What Is Whipple Disease?

Whipple Disease
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Whipple disease is among the rare bacterial infections that particularly affect the gastrointestinal system and joints.

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It was named after George Whipple, who was the first to uncover the disease in 1907.

Historical Background on Whipple Disease

In 1907, May 9th, George Whipple, who was a pathology instructor at Johns Hopkins University then, performed an autopsy on a middle-aged physician who had been living at Istanbul, Turkey.

He was reported to have presented gradual weight loss, loss of strength, excessive stooling, consisting mainly of neutral fat and fatty acids, undefined abdominal signs and arthritis in multiple joints.

The findings of the autopsy consisted of;

  • Polyserositis- a chronic inflammation of several serous membranes with effusions in serous cavities, resulting in fibrous thickening of the serous membranes (a smooth protective tissue membrane that surrounds organs).
  • Aortic valve lesions- Abnormal damages to the Aortic valve
  • Prominent disposition of fat with intestinal walls and mesenteric lymph nodes
  • Infiltration of the intestinal walls and mesenteric lymph nodes by foamy macrophages
  • Presence of rod-like bacilli.

But Dr. Whipple did not consider the rod-like bacilli to be the etiology of the disease. In 1961, this rod-shaped structures in the intestinal mucosa and intestinal macrophages were demonstrated by laboratory electron microscopy examination to have the structural features of bacteria.

So many attempts were made in time past to identify the causative organism for Whipple’s disease, isolation of variety of species in pure culture from intestinal biopsy specimens including Steptococcus, Corynebacterium and Haemophilus.

In 1992, it was finally confirmed from results of several medical research that the causative bacterium (not yet validated then) of Whipple’s disease was “Tropheryma whipplii”.

In 2000, it was acknowledged from series of several investigations, Whipple’s contribution and to allude to the malabsorption caused by the disease and it was reported, successful isolation and establishment of a strain of Whippl’s disease bacterium obtained from the valve of a patient’s blood.  

Facts About Whipple’s Disease

Over time, thanks to numerous medical researches more knowledge has been brought to light about the Whipple’s disease.

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Some well-known bullet facts about the Whipple’s disease include;

  • Whipple disease are rare disease, but it can be life threatening and less than 1000 cases have been reported till date.
  • Whipple disease js associated with the bacterium called Tropheryma whipplii.
  • Whipple’s disease has been considered for a long time to be a gastrointestinal disease but recently, clinical manifestations of Whipple’s disease are myriad and nonspecific.
  • Whipple’s disease may affect the central nervous system, lungs, heart eyes, skin and joint. These organs may be the first manifestation of the disease.
  • Males are more frequently affected by whipple’s disease than females. That is, 80% of related cases are males.
  • Although all age groups can contract the whipples disease, but it is known that those between 40-50 years are at a higher risk and children are only extremely rarely affected.
  • Whipple’s disease occurs around the world, but most patients are Caucasian. This supports some speculations that, a certain genetic predisposition could be involved, and that some kind of immune defect may predispose individuals to the development of the disease.
  • Whipple’s disease might be associated with immunogenetic factors, as two sets of brothers, a brother-sister pair and a father-daughter pair with the disease has been reported, suggesting the Whipple’s disease might be familiar.
  • Diagnosis of Whipple’s disease is complicated because its symptoms are very similar to other common conditions like Neurological disorders and celiac disease. Diagnostic tests carried out include, Endoscopic small-bowel biopsy, polymerase chain reaction test and some blood tests.

Causes of Whipple’s Disease

The disease is closely associated with the T. whipplii bacteria. The bacteria cause sore and internal thickening of soft internal tissue layers of organs, primarily the intestine, causing the lining mucosa wall to swell and thicken (lesions).

This is as a result of the villi (finger-like tissues along the intestinal wall that carry out absorption in the intestine) thickening and losing their natural shape.

This destroys the villi and prevents them from effectively absorbing nutrients leading to many of the Whipple’s disease symptoms. Whipple’s disease infection can spread to the heart, lungs, joints and brain and even affect any system of the body.

Individuals most likely to contract the disease are those with decreased ability to break down proteins. How the bacteria get to the body is not particularly known but suggestions were made known that it could be from contaminated food and water.

Signs and Symptoms of Whipple’s Disease

Whipple’s disease has its symptoms widely spread among patients but the most common sign of whipple’s disease is weight loss as a result of malabsorption of nutrient through the bloodstream and diarrhea.

Other symptoms include:

  • Abdominal pain
  • Abdominal bleeding
  • Skin darkening
  • Steatorrhea (Oily stool)
  • Fever of unknown origin
  • Loss of Appetite
  • Body wweakness
  • Anemia 
  • Joint Pain

Clinical manifestations of Whipple’s disease that do not occur frequently but can serve as indicators include:

Neurological symptoms like;

  • Abnormal eye movement,
  •  Facial muscle abnormalities,
  • confusion,
  • seizures (epilepsy),
  •  Vision impairment.
  •  Inflamed lymph nodes
  • Cachexia

Ophthalmological symptoms like:

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  • Uveitis (inflammation of the uvea of the eye)
  • Retinitis (inflammation of the Retina)
  • Keratitis (inflammation of the cornea)
  • Optic neuritis (inflammation of the optic nerves)
  • Papiloedema- swelling of the optic disc as a result of increased intracranial pressure

Cardiovascular manifestations:

  • Endocarditis (inflammation of the heart valve)
  • Pericarditis (inflammation of the pericarduim)
  • Myocarditis (inflammation of the myocarduim)
  • Coronary arteritis (inflammation of the coronary artetry)
  • Sudden death
  • Pleural effusion – a buildup of fluid between the tissues that line the lungs

Treatment of Whipple’s Disease  

Whipple’s disease has been considered for a long time, a fatal primary metabolic disorder, but recently, patients have greater chances of survival with current treatments.

Antibiotics are the primary treatment for Whipple’s disease as they prevent symptoms from becoming fatal.

Antibiotics may include:

  • Penicillin
  • ampicillin
  • Streptomycin
  • Erythromycin
  • Doxycycline with hydroxychloroquine

These antibiotics may be used in combination, administered orally or through IV in the arm to kill the bacteria for 2 weeks. In addition, you will likely be on daily antibiotics for one to two years depending on the severity of the presented symptoms.

Fluids may also be recommended by your physician to keep you hydrated and extra vitamins and minerals to ensure your body gets enough nutrients.

Some other treatment option are; taking antimalarial medication for 12 to 18 months, using iron supplements to assist with anemia, maintaining a high calorie diet to help with nutrient absorption, administration of corticosteroids to help ease inflammation, taking non-steroidal pain relievers like ibuprofen.

On a long-term outlook, many symptoms will disappear within a month after treatment commences. The most affordable advise you can exercise is to continue to take your antibiotics as relapses are common. 

Conclusion

Whipple’s disease is a bacterial infection that can lead to death, if not treated properly or immediately. Though there is no known way of preventing Whipple’s disease, however, practicing good hygiene like regularly washing of hands and drinking clean water can minimize the risk of contracting the bacteria.

Sources;

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Whipple Disease
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Ehikioya Hope
making my mark in the digital world, one post at a time.
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