Whipple’s disease is a very rare infectious disease of the intestines, which, however, can also affect all other organs of the body. Little is known about the pathogenesis of the disease. Left untreated, Whipple’s disease is fatal.
What is Whipple’s disease?
Whipple’s disease, also known as Wipple’s disease or intestinal lipodystrophy, is an infectious disease of the small intestine caused by the pathogen Tropheryma whipplei. The disease was first described by the American pathologist George Hoyt Whipple (1878 – 1976). The causative bacterium was only discovered after his death in 1991 and 1992. See fun-wiki for What is BSE used for.
Starting in the gut, the disease develops into a systemic condition involving many other organs. Without treatment, the prognosis is very poor. It almost always ends fatally due to general organ failure . However, the disease is very rare. So far, only about 1000 cases have been described worldwide.
The disease most commonly occurs between the ages of 40 and 55, although other age groups can also be affected. Only children and young people seem to be spared the infection. It has been found that men are eight times more likely than women to develop Whipple’s disease. The reason for this is not known. Most cases have been detected in rural areas. There is no transmission of the infection through contagion.
Causes
The cause of Whipple’s disease is infection with the bacterium Tropheryma whipplei. The pathogen probably enters the intestine via oral intake. Despite its frequency, however, very few people develop Whipple disease. This may be due to the fact that a genetic defect is a prerequisite for triggering it. The pathogen is phagocytosed by macrophages. But the macrophages remain in the mucous membrane and create a lymphatic congestion. The mucous membrane of the small intestine swells considerably.
In investigations, it was determined by PAS staining that the macrophages contain crescent-shaped inclusion bodies. The phagocytic macrophages are also found in other organs. The cause of the lymph blockage is probably the incomplete breakdown of the trapped pathogens. Therefore, a genetic defect is suspected, which limits the function of the macrophages. In addition, the antigen variant HLA-B27 was found very frequently in the patients.
Symptoms, Ailments & Signs
Whipple’s disease is characterized by a variety of different symptoms. The disease is primarily an infection of the small intestine, which later spreads to the entire body. Therefore, a distinction is made between intestinal and extraintestinal symptoms. The intestinal symptoms are characterized by abdominal pain, foul-smelling stools, fatty stools , diarrhea, meteorism, and weight loss, among others.
Typical malabsorption features occur with nutrient, vitamin and mineral deficiencies, muscle weakness, anemia and mucosal changes. Extraintestinal symptoms include fever, lymph node swelling, enteropathic arthritis, heart failure or even dementia due to central nervous system involvement.
The disease is progressive and always fatal if left untreated. Even after treatment, recurrences can still occur years later. The intestine is then usually no longer affected, but the central nervous system and the brain. Therefore, the relapses occur mainly in the form of neurological deficits.
Diagnosis & course of disease
Whipple’s disease is diagnosed via an endoscopic clarification. There are numerous white lymphatic vessels that are congested. On the recordings it looks like a snow flurry. Furthermore, a biopsy is carried out, in which the SPC cell detection is carried out using PAS staining. Radiologically, a palisade-like enhancement of the Kerckring folds of the small intestine is detected.
When examining the stool, the pathogen Tropheryma whipplei must be found for the final diagnosis. This detection of the pathogen is considered the only conclusive evidence for the disease. In order to be able to assess the involvement of the internal organs, imaging procedures such as X-ray examinations of the intestine, sonography and CT of the abdomen, magnetic resonance imaging of the brain or ultrasound examination of the heart are carried out.
Complications
Whipple’s disease must always be treated by a doctor. If left untreated, this disease can, in the worst case, lead to the death of the patient. Those affected primarily suffer from complaints of the intestines and stomach. This often leads to a fatty stool and pain in the abdomen.
In addition, the stool is often foul-smelling and there is a relatively strong weight loss. Whipple’s disease also leads to general fatigue and muscle weakness in those affected. The patients suffer from vitamin and mineral deficiencies, which in general can have a very negative impact on the patient’s health.
Heart failure can also result in death. Symptoms of dementia can also occur and significantly reduce the patient’s quality of life. Self-healing of Whipple’s disease does not usually occur. Furthermore, the motor skills of those affected are also restricted by the disease.
The treatment of Whipple’s disease is carried out with the help of antibiotics and is successful in many cases. However, complications arise when treatment is started too late and the pathogens have already spread to other organs. In most cases, the patients are then dependent on lengthy therapy.
When should you go to the doctor?
A doctor should be consulted as soon as persistent or repeated digestive irregularities occur. The affected person needs help with fatty stool, constipation or diarrhea. If you experience abdominal pain or an unusual noise development within the digestive tract, a doctor’s visit is advisable. Unwanted weight loss is always a warning signal from the organism.
It must be clarified by a doctor so that the affected person does not develop an acute health-threatening condition. A decrease in muscle strength, reduced performance and low physical resilience must be examined and treated. An increased body temperature, swelling of the lymph and disturbances in the heart rhythm indicate irregularities that need to be examined by a doctor.
A doctor should be consulted if there are losses in mental skills, problems with orientation or concentration, or if memory is impaired. Pain in the joints or limitations in mobility are worrying and indications of an existing disease with a need for action. If the existing symptoms increase in scope and intensity or if further irregularities appear, a doctor should be consulted. Since Whipple’s disease can lead to premature death without medical treatment, a doctor should be consulted at the first sign of a discrepancy.
Treatment & Therapy
Antibiotics are used to treat Whipple’s disease. Penicillins, sulfonamides, tetracyclines, cephalosporins or macrolides have proven particularly useful. After a week of treatment, many symptoms such as diarrhea and fever disappear. However, it has been shown that Whipple’s disease often recurs, although neurological deficits usually occur. This suggests incomplete elimination of the pathogen.
The pathogen finally settles in almost all organs and is therefore becoming increasingly difficult to reach with antibiotics. Due to the few cases of disease, there is also little experience of completely combating it. Therefore, the antibiotic therapy was extended to at least a year in the hope of being able to reach all pathogens. Conclusive experiences have not yet been made with it.
Above all, it is unclear whether the pathogens in the brain can also be fought with it. Cases have been reported in which neurological symptoms reappeared after several years. In parallel with antibiotic treatment, at the beginning it is necessary to gradually build up the body. Vitamins, minerals and trace elements must be administered systematically for this purpose.
In order to gain long-term experience, the success of the therapy is constantly monitored through regular follow-up examinations. This is done by further control endoscopies. These follow-up examinations should be carried out for at least ten years.
Outlook & Forecast
The prognosis for Whipple disease depends on the treatment. This wasting of the body through malabsorption and weight loss is fatal if left untreated. However, it takes a very long time before a healthy person is so weakened by this infection that he dies. This leaves room for treatment approaches.
The prospect of successful treatment depends largely on the antibiotics and any resistances. If treated properly, the infection can be successfully combated. The patient then has to rebuild his body, which makes good nutrition essential.
The danger with Whipple’s disease is not so much the clinical picture, which is considered to be easily treatable. Rather, a late diagnosis often has a negative effect on the prognosis. It is also possible for the disease to break out again years after treatment. This is then due to the bacterial populations that are still present, which have usually settled in the brain and are not accessible for therapies there. Such relapses often lead to neurological symptoms.
While the symptoms caused by Whipple’s disease are very serious and can be fatal, the prognosis for treated Whipple’s disease is very good.
Prevention
So far, no recommendations for the prevention of Whipple’s disease can be given, especially since there must be a genetic predisposition for the pathogenesis of the disease. The pathogen is found everywhere and enters the body through food.
Aftercare
Most of those affected with Whipple’s disease have very few and usually only very limited follow-up measures available. For this reason, the person affected by this disease should ideally see a doctor very early on, so that other complications and symptoms do not arise. In the worst case, the affected person can die if Whipple’s disease is not treated properly or is not recognized until late.
Since this is a genetic disease, a complete cure is usually not possible. If the person concerned wants to have children, they should have a genetic examination and counseling carried out so that the disease cannot recur in their descendants. As a rule, those affected are dependent on taking medication to permanently limit the symptoms.
It is often necessary to take antibiotics, but the person concerned should not drink alcohol while taking them. In general, a healthy lifestyle with a balanced diet also has a very positive effect on the further course of the disease. Despite treatment, many of those affected have to reckon with a reduced life expectancy due to Whipple’s disease.
You can do that yourself
Patients affected by Whipple disease may be treated with different antibiotics for a long time. This treatment should also not be questioned by the patient or broken off by the patient themselves, as the disease can otherwise be fatal. However, a long antibiotic therapy also has the disadvantage that it not only kills dangerous germs and bacteria, but also healthy ones.
This also applies to the germs that are in the intestines and contribute to a well-functioning immune system. For a healthy intestinal flora, Whipple’s disease patients can take living microorganisms during their antibiotic treatment and beyond, which are available as so-called probiotics in pharmacies. They are intended to compensate for the loss of germs caused by antibiotics. Also less stress, a regular life without nicotine and alcohol, but with a lot of exercise and enough sleep can promote healing. The doctor treating you may prescribe vitamins and minerals to compensate for the vitamin and mineral deficiencies that have occurred during the illness.
Of course, a conscious lifestyle, which includes a healthy, low-sugar diet, also contributes to this. It should contain as many fresh, vitamin-rich foods as possible, such as fruit and vegetables, plus lean meat, eggs and fiber from natural whole grain products such as oatmeal, and omega-3 fatty acids from valuable linseed or fish oil.
