Whipple's disease is a systemic infectious disease of the small intestine that occurs in individuals with impaired T-cell immunity and macrophages unable to digest bacteria or bacterial antigens.
Content
Causes of the disease
The cause of the disease remains unclear. An electron microscope examination revealed bacteria or products of phagocytic activity, which were also of bacterial origin.
Cells containing these substances are found in this disease not only in the small intestine, but also in other organs and tissues. This explains the damage to the entire body.
It was not possible to accurately identify the resulting bacilli.
However, persistent suppression of T-cell immunity, which persists even after treatment, indicates that the main cause of the disease is the great importance of the reactivity of the macroorganism.
Whipple's disease occurs 5 times more often in men than in women. The disease is rare; rural residents and farmers are more likely to suffer from it.
Symptoms
Most patients experience
- diarrhea 3-8 times a day,
- polyfecal,
- flatulence,
- lack of appetite,
- weight loss,
- cramping pain in the upper abdomen,
- hidden bleeding.
Symptoms not related to the gastrointestinal tract are very varied. The most typical are
- inflammation of the retroperitoneal, less often peripheral, lymph glands,
- inflammation of the joints,
- periodic fever.
- Less commonly observed
- skin pigmentation,
- myocarditis,
- peritonitis,
- pleuropericarditis,
- amyloidosis.
Neuropsychiatric syndromes are sometimes observed:
- memory loss,
- sleep disturbance,
- behavior disorder
- damage to the oculomotor nerves (ophthalmoplegia),
- involuntary high-frequency oscillatory eye movements (nystagmus),
- facial nerve paresis,
- seizures,
- meningoencephalitis.
At the onset of the disease, symptoms such as
- abdominal pain,
- joint pain (in 50% of cases in the knee joints),
- spine pain,
- cough,
- low-grade fever.
As the disease develops, diarrhea, steatorrhea (diarrhea with a large amount of fat in the stool), and weight loss appear.
During this period, damage to other organs also occurs:
- enlarged lymph nodes,
- skin rashes,
- expanding the boundaries of the heart,
- heart murmurs,
- heart rhythm disturbance,
- myocardial damage.
Diagnostics
The diagnosis is established on the basis of histological studies of the mucous membrane of the small intestine.
Material for research is obtained during duodenoscopy.
During duodenoscopy, redness, swelling and sharp thickening of the intestinal walls are observed.
Electron microscopy reveals a large number of small gram-positive bacilli in the mucous membrane of the small intestine, which disappear with repeated biopsies after long-term treatment with antibacterial drugs.
PAS-positive macrophages are found in Whipple's disease and in lymph nodes, cardiac muscle, lungs, liver, and nerve trunks.
Differential diagnosis
In case of Whipple's disease, differential diagnosis is carried out with the following diseases:
- lymphoma,
- Crohn's disease of the small intestine,
- gluten intolerance (celiac disease),
- amyloidosis,
- intestinal tuberculosis.
An accurate diagnosis of Whipple's disease can be made by biopsy of the mucous membrane of the subbulb of the duodenum.
Treatment
Treatment of the disease is long-term. It is based on antibacterial therapy with tetracycline antibiotics. The drugs are used intermittently.
The following drugs are used in the form of monotherapy or with the replacement of one drug with another:
- tetracycline,
- ampicillin,
- penicillin with streptomycin intramuscularly,
- ceftriaxone,
- chloramphenicol,
- trimethoprim,
- sulfamethoxazole,
- cefixime
A number of foreign clinicians have come to the conclusion that the best effect is achieved when taking trimethoprim in combination with sulfamethoxazole, i.e. the drug Biseptol, which contains both active substances.
With treatment, fever and joint symptoms disappear after a few days, diarrhea - no later than after 2-4 weeks.
PAS-positive macrophages in the small intestinal mucosa persist for a long time. Even after long-term treatment, a relapse or resistance to the chosen drug may develop.
On average, the course of treatment lasts 2-5 months. It is determined by the results of repeated studies and the patient’s condition.
Along with taking antibacterial drugs, metabolism is corrected. Due to poor absorption of fats, ordinary fats are replaced by taking fat enpit and portalac.
Relapse Prevention
Patients should be under constant supervision of a gastroenterologist. A repeat biopsy should be performed 2-4 months after the start of treatment, then once a year after its completion.
Periodic examinations will help prevent relapses, since often re-infection of the small intestinal mucosa does not begin to show symptoms until several months later. The threat of relapse can be eliminated by timely administration of an antibacterial drug.
During the period of remission, the performance of patients is not reduced. The issue of disability is decided in each case individually and depends on the frequency of exacerbations.
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