Whipple's disease
Contents:
Description:
Whipple's disease — a rare disease of intestines of the infectious nature with various clinical manifestations. It is for the first time described American the pathologist by George Whipple (English George H. Whipple) in 1907 [1] observing in autopsiyny material the expressed increase in mezenterialny lymph nodes in combination with an inflammation of serous covers. At a histologic research of intestines and lymph nodes it revealed multiple deposits of lipids and a large number of the macrophages supporting argyrophil palochkoobrazny structures. The author assumed that fat metabolism disturbance which he suggested to call "an intestinal lipodystrophy" is the cornerstone of a disease.
Symptoms of the Disease of Whipple:
Whipple's disease men at the age of 40-50 years have, however it is not excluded also in other age categories. The disease affects various bodies and systems.
In a clinical picture of a disease malabsorption symptoms prevail. During a disease allocate 3 stages:
I \abenteric symptoms (fever, polyarthritis);
II \diarrhea, loss of body weight, proteinaceous insufficiency, symptoms of hypovitaminosis, disturbance of nutritive absorption.
III \system manifestations (neurologic symptomatology, pancarditis, polyserositis)
The following abenteric displays of a disease of Whipple are possible:
Symptoms of adrenal insufficiency — the low arterial pressure, a xanthopathy, anorexia, a hyponatremia, a hypoglycemia.
Damage of skin — an erythema, a knotty erythema.
Damage of eyes — a uveitis, a retinitis, a keratitis.
Defeat of cardiovascular system — a fibrous endocarditis, myocarditis, a pericardis, a polyserositis, a coronaritisis.
Defeat of the central and peripheral nervous system — a hearing disorder, sight, an ataxy, damage of cranial nerves, polyneuropathy.
Etiologies of Whipple:
George Whipple the first found the microorganisms similar to a pale spirochete in a mucous membrane of a small bowel. However, a long time many scientists disputed the infectious nature of this disease. In 1949 it was established that from lymph nodes and a small bowel contain in bioptata PAS positive (English PAS — periodic acid Schiff) macrophages with the inclusions similar to decomposition products of bacteria. Disease-producing factors many bacteria allocated in culture from a bioptat appeared (korinebakteriya, brutsellopodobny microorganisms, a streptococcus L-form, etc.). In 1991 R. Wilson, and then in 1992 allocated to D. Realman by means of the polymerase chain reaction (PCR) a gram-positive bacillus from material of the infected patient's tissues, the called Tropheryma whippelii (from the Greek trophe — food, eryma — a barrier).
T. whippeli is a gram-positive bacterium. On the basis of similarity of 165 sequences of ribosomal RNA, her relationship with actinomycetes of group B (Cellulomonadaceaea family) is established. The bacterium has a three-layered cellular cover, the only circular chromosome and the small size of a genome. Believe that not everything can be pathogenic, and strictly certain strains of the activator. Tropheryma whippelii was cultivated in the cellular environment of the human phagocytes deactivated by Interlaken (IL-4, IL-10) and dexamethasone.
Treatment of the Disease of Whipple:
Treatment has to include the bactericidal antibiotics which are well getting through a blood-brain barrier, for example tetracycline or its analogs doxycycline, metacycline, тайгециклин within 2-5 months. Then for maintenance of remission it is necessary to pass on intermittent (up to 9 months) therapy with administration of drug every other day or 3 days a week with a break in 4 days.
For treatment of a disease of Whipple Trimethoprimum sulfamethoxazole (Biseptolum, Co-trimoxazole) is also used.
At cerebral defeats the following scheme of therapy is recommended: 1,2 mln units of benzylpenicillin (penicillin G) and 1 g of streptomycin parenterally daily within 2 weeks with the subsequent purpose of Co-trimoxazole (Trimethoprimum — 160 mg and sulfamethoxazole — 800 mg) twice a day within 1-2 years to a negative take of PTsR and disappearance of T. whipplii in bioptata from a duodenum.
Long, within 1-2 years, the maintenance therapy is considered Co-trimoxazole the most successful in the plan of prevention of neurologic symptomatology.
As auxiliary therapy purpose of corticosteroids of 30-40 mg/days with the subsequent gradual dose decline, correction of metabolic and water and electrolytic frustration, completion of a vitamin deficiency is considered.
