Whipple's disease: diagnosis

Whipple's disease can be suspected on the basis of anamnesis, clinical manifestations, laboratory, endoscopic and radiographic data. Cardinal clinical manifestations of the disease are diarrhea, pain in mesogastric, increasing weakness, weight loss, polyarthritis (or arthralgia), and lymphadenopathy.

Laboratory data

1. The general analysis of a blood: an anemia (more often hypochromic in connection with deficiency of iron, less often - hyperchromic, macrocytic, caused by a violation of absorption of vitamin B ₁₂ ); hyperleukocytosis; sometimes eosinophilia, more often thrombocytosis; an increase in ESR.
2. The general analysis of urine: at a serious current of a malabsorption syndrome the small proteinuria is possible.
3. Coprologic analysis: polyphecal, steatorrhea; possibly the appearance of undigested muscle fibers, sometimes determined by hidden blood.
4. Biochemical analysis of blood: a decrease in the total protein, albumin, calcium, iron, potassium, sodium, prothrombin, glucose (not in all patients), increased bilirubin, aminotransferase.
5. Suction function of the small intestine is reduced.

Instrumental data

• X-ray examination. In the study of the small intestine, the expansion of the small intestine loops is determined, its contours are coarse, and there may be a violation of marginal filling defects with enlarged mesenteric lymph nodes. The folds of the small intestine mucosa are enlarged, it is unevenly thickened (the "granularity" pattern) due to infiltration. Elasticity of the small intestine wall is significantly reduced. With an increase in retroperitoneal lymph nodes, an expansion of the duodenal bend is possible.
• Ultrasound of the abdominal cavity organs. It can reveal an increase in mesenteric, parapancreatic, retroperitoneal lymph nodes.
• Lymphography. Identifies nonspecific inflammatory changes in retroperitoneal lymph nodes, as well as signs of subdiafragmal stasis of lymph.
• Biopsy of the small intestine mucosa. At present, biopsy of the small intestine mucosa is the only method that allows verifying the diagnosis of Whipple's disease. A biopsy is performed during endoscopy of the small intestine in the area of the duodenal transition. This area of the small intestine is affected in all patients, both in the early and late stages of the disease. Histological evidence of Whipple's disease is the following signs, revealed in biopsies:
  • infiltration of the own layer of the mucosa of the small intestine by large PAS-positive macrophages ("foamy" macrophages); these granules in macrophages are detected by coloring with diamond fuchsin. Macrophages can also be detected in biopsies of other organs - lymph nodes, spleen, liver;
  • detection in biopsies using electron microscopy bacilliform Whipple bodies, which are cells in the form of rods (1-2 μm x 0.2 μm) with a three-layered shell. They are defined in the intercellular space and are also present inside macrophages. PAS-positive macrophage material is a lysosomal material containing bacteria at different stages of destruction;
  • intra- and extracellular accumulation of fat in the mucosa of the small intestine, as well as in mesenteric lymph nodes;
  • enlargement of lymphatic vessels.
• FEGDS. There are signs of chronic gastritis, duodenitis.
• Determination of the content of hormones in the blood. In severe malabsorption syndrome, blood levels of cortisol, thyroxine, triiodothyronine, and sex hormones decrease.

Differential diagnosis. Clinical triad - diarrhea, loss of body weight and increasing weakness - requires differential diagnosis, primarily with a malignant tumor of the gastrointestinal tract, chronic inflammatory bowel disease, sprue, Zollinger-Ellison syndrome.

With the help of endoscopic and radiologic examination of the gastrointestinal tract, cancer, Crohn's disease, ulcerative colitis can be excluded. The absence of recurrent ulceration in the upper parts of the digestive tract, gastric hypersecretion and hyperacidity, hypergastrinemia, pancreatic tumor with ultrasound or computed tomography allows to reject Zollinger-Ellison syndrome.

In differential diagnosis with Whipple's disease, Addison's disease should also be excluded. To make the correct diagnosis help the laboratory data - hormonal studies, the detection of hyperkalemia in Addison's disease, signs of thickening of the blood, lack of steatorrhea.

Whipple's disease should be suspected in patients with arthralgia or arthritis in the event of diarrhea, malabsorption or unexplained weight loss, especially if one can not determine the type of arthritis. With Whipple's disease, tests for the rheumatoid factor are negative or weakly positive. Rheumatic tests are negative. The level of uric acid in the serum is normal.

Fever often outstrips the development of intestinal manifestations. With fever of unknown origin, one must also bear in mind the possibility of developing this suffering.

In patients with Whipple's disease, fever, lymphadenopathy is common, and in the abdominal cavity tumor formation can be determined. In this regard, it is necessary to exclude lymphoproliferative disease, primarily lymphogranulomatosis.

The final diagnosis of Whipple's disease in all cases is based only on the histological examination of the affected organs, primarily the small intestine.