Definition
Whipple's disease, also called intestinal lipodystrophy, is a rare chronic systemic disease caused by the bacterium Tropheryma whippelii .
The infection is responsible for an accumulation of lipid and glycoprotein material in the intestinal lymphatic vessels and in the lymph nodes. The most affected site is the mucosa of the small intestine, but other organs are also involved, including the spleen, heart, lungs, liver, kidneys, eyes and organs of the central nervous system, in addition to the joints.
Conditions that promote infection are not yet known, but an acquired or genetic immune predisposition is suspected. Whipple's disease predominantly affects men aged 30-60 years.
Tropheryma whipplei has been found, in particular, in wastewater treatment plants
.
Most common symptoms and signs *
- Anemia
- Anorexia
- Cachexia
- Conjunctivitis
- Dementia
- Diarrhea
- Yellow Diarrhea
- Skin discoloration
- Abdominal pain
- Chest pain
- Articolar pains
- Muscle pains
- Edema
- Temperature
- Glossitis
- undernourishment
- Swollen lymph nodes
- Ophthalmoplegia
- Dark skin
- Weight loss
- Joint stiffness
- steatorrhea
- Cough
Further indications
The symptomatology of Whipple's disease varies depending on the affected apparatus.
Usually, the first manifestations are the low-grade fever, joint pain (polyarthralgia) and arthritis. Subsequently, Whipple's disease manifests itself with symptoms associated with intestinal malabsorption: steatorrhea, loss of appetite, watery diarrhea, abdominal pain and progressive loss of body weight. Other manifestations include anemia, lymphadenopathy, peripheral edema, increased skin pigmentation, chronic cough and pleuritic pain.
Sometimes, cardiac symptoms (eg endocarditis and pericarditis), hepatic and neuropsychiatric (cognitive dysfunction, ophthalmoplegia and clonic contractions of facial musculature) are also possible.
If left untreated, Whipple's disease is progressive and leads to disability due to central nervous system involvement and / or decay death.
The diagnosis is formulated by a histological examination (lymph node or bowel biopsy), which shows the presence of the causal bacterium in the tissues and specific alterations. Culture tests and molecular genetic analyzes, such as PCR (polymerase chain reaction), on samples of intestinal tissue, cerebrospinal fluid, lymph nodes or synovial fluid, may be useful for confirmation.
Treatment involves the use of antibiotics such as tetracycline, chloramphenicol, chlortetracycline, sulfasalazine, ampicillin and penicillin, or the combination trimetroprim / sulfamethoxazole for at least 1 year. Usually, antibiotic therapy is associated with good results. Clinical improvement is rapid, with resolution of fever and joint pain in a few days. Intestinal symptoms generally disappear within 1 to 4 weeks, although histological healing may occur after 2 years. Fallbacks are not excluded.
