Whipple’s Disease

What is Whipple’s Disease?

Whipple’s disease (intestinal lipodystrophy) is a rare disease manifested by migrating arthritis and progressive lesions of the small intestine (diarrhea, steatorrhea – “fatty stools”, in severe cases, impaired absorption syndrome). The onset of the disease is usually observed at the age of 30-50 years, with men getting sick much more often than women (9:1).

Pathogenesis During Whipple’s Disease

In the past, Whipple’s disease was considered as a primary violation of fat metabolism. Currently, there is evidence that this is apparently a systemic infection that can affect any organs or tissue, although it is mainly manifested in the pathology of the joints and small intestine.

In the mucous membrane of the intestine, synovial tissue, as well as in virtually every organ, electron-microscopic examination of rod-like inclusions of microorganisms that disappear after treatment with antibiotics can be detected. The most important diagnostic feature of this disease – the presence of multi-core foam cells in the mucous membrane and lymph nodes of the affected intestine, macrophages containing CHIC-positive (glycoprotein) granules – is explained by the incomplete phagocytosis of these bacteria, which are called Whipple bacilli. However, to obtain a culture of the microorganism in the laboratory has not yet succeeded.

Symptoms of Whipple’s Disease

Years and even decades may pass before the onset of the classic symptoms of intestinal damage. Prodromal events can relate to changes in almost any organ, but small fever, loss of body weight not associated with diarrhea, skin hyperpigmentation, lymphadenopathy, hypotension, a tendency to develop polyserositis, etc. are most typical. Most frequent prodromal syndrome is intermittent and sometimes long-term non-erosive arthritis, not differing from arthritis in other enterogenous infections; sometimes revealed sacroiliitis;

Anemia and leukocytosis with neutrophilia are detected in the blood. A characteristic feature of synovial effusion is a high content of mononuclear cells. Characteristic features of the advanced stage of the disease: fever, weight loss, diarrhea, deterioration of the processes of absorption in the intestine.

In past years, before the use of antibiotics, the disease ended in death; antibiotic therapy is currently interrupting the course of the disease.

Diagnosis of Whipple’s Disease

Before the appearance of pathological changes in the gastrointestinal tract, the diagnosis is difficult to make. In the presence of systemic manifestations, the process is mistaken for rheumatoid arthritis, sarcoidosis, Addison’s disease, etc. At the stage of intestinal lesion, the diagnosis becomes quite simple. In some cases, it is necessary to use radiography of the small intestine, laparotomy with a biopsy of the lymph nodes, or oral biopsy of the mucous membrane of the small intestine to detect characteristic histological changes.

Treatment of Whipple’s Disease

Penicillin is prescribed (1 000 000-2 000 000 U/day) and streptomycin (1 g/day) for 10-14 days, and then tetracycline (0.5-1 g/day) during the year, since the termination treatment at an earlier time may result in the resumption of the process.

Non-hormonal anti-inflammatory drugs are used symptomatically; from the appointment of corticosteroids are currently abstaining.

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