Whipple's Disease

Clinical symptomatology and diagnosis. Among the patients suffering from the disease Whipple, you can often find men aged 30 to 60 years, marking gradually progressive weakness, diarrhea, weight loss and joint pain without their deformation.
The earliest symptom is arthralgia observed in 3/4 of the cases and usually within a few years precede the development of gastrointestinal disorders. Marked pains in the small and large joints, rarely is redness and swelling. In the future, the symptoms of acute lesions of the gastrointestinal tract, usually accompanied by the appearance of the expressed pain in the joints.
Later there bloating, cramping around the navel, sometimes in combination with nausea and vomiting; as a rule, patients suffer from anorexia and cytopenia. Gradually against the background of gastrointestinal discomfort appear diarrhea that become the leading complaint of patients. The chair is from 5 to 15 times a day, watery, rich. In some cases there steatorrhea. Intestinal bleeding is rare when Whipple's disease.
Patients may increase the temperature. In some cases, there is a dry cough, shortness of breath. Taking into account the systemic nature of the disease, diagnosis is sometimes even in the absence of gastrointestinal disorders.
Objective examination noteworthy sudden exhaustion, weakness, skin sometimes pigmented, gets gray-brown color, resembling the color of the skin of patients with edisonova disease. In later stages of the disease are revealed swelling. Often able to probe increased peripheral lymph nodes. For some patients are exudative pleuritis, and pericardial effusion, in other cases may listens murmurs as manifestations of endocarditis, often find hypotension as a manifestation of secondary addisonizme. Abdomen is usually stretched, diffuse tense, palpation can be determined poorly limited "tumor" in the upper abdomen. The spleen and liver are rarely raised.
When illness Whipple there are no pathognomonic changes laboratory and biochemical parameters. Often hypochromic, microcytic anemia, blurred marked leukocytosis.
In severe cases, there hypoproteinemia, sharp malabsorption D-xylose, radioactive albumin, carotene and vitamin a, fatty acids. In the coprogram often found neutral fat and fatty acids.
X-ray study of the small intestine visible thick folds, and in some areas the relief is smoothed, there is a "sprinkling" of barium suspension or "cluster" in some places.
Crucial to the diagnosis has aspiration biopsy of the small intestine or peripheral lymph node. Usually find specific macrophages - PAS-positive "frothy" cells.
The diagnosis of Whipple disease is based on polymorphous clinical symptoms (fever, lymphadenopathy, steatorrhea, polverosa, cachexia, signs of addisonizme) in combination with the results of in vivo imaging. In difficult unclear cases resort to diagnostic laparotomy.
The forecast. Until recently, Whipple's disease refers to diseases and adverse, fatal within 1-2 years. Describes the cases and with a longer period (up to 61/2 years), some patients experience short-lived spontaneous remission (Palmer and others, 1950).
If you define the duration of the disease from the moment of occurrence of artralgia, it often reaches 8-10 years.
Treatment. Most researchers are supporters of combination therapy with antibiotics and steroid hormones on the background of a high-calorie diet rich in protein. In cases with steatorrhea justified the appointment of a diet without gluten. Appropriate parenteral introduction of fat-soluble vitamins A, D and K, preparations of iron and calcium.
Antibiotics (including a wide spectrum of action) are used in large doses in the form of courses - penicillin 1-2 million UNITS, streptomycin - 1 year, tetracycline for 1-1,5 million IU per day.
Steroids in exchange assignment in normal doses sometimes have a positive effect, cause a temporary remission, but change the course of the disease and limit the progression of these drugs cannot.
However, Chears and others (1964) received more encouraging results in concurrent administration of large doses of antibiotics and steroid hormones.
In the light of data Trier, suggesting a possible bacterial nature Whipple's disease, use of antibiotics is becoming increasingly validity.