Amyloidosis - is a systemic disease characterized by deposition of amyloid in the tissues (a complex protein-polysaccharide complex).
Amyloidosis leads to atrophy, multiple sclerosis, and multiple organ failure.
The frequency of - no less than 1:50 000 (mostly occurs after age 60).
Classification
1. Primary amyloidosis is caused by changes in cells at multiple myeloma, Monoclonal hypergammaglobulinemia, Waldenstrom's macroglobulinemia.
Amyloid is composed of light chain immunoglobulins, their synthesis is sharply increased at the indicated diseases.
2. Secondary amyloidosis occurs due to chronic inflammatory diseases (e.g., rheumatoid arthritis, Osteomyelitis, bronchiectasis, malaria, Tuberculosis, leprosy).
Amyloid is composed of fibrillar amyloid protein and its decay products - serum amyloid A protein.
3. Family (idiopathic) amyloidosis. Usually - a congenital defect of enzymes. There are several forms of congenital amyloidosis, such as Mediterranean fever (family paroxysmal polyserositis - a disease of unknown cause, manifested by abdominal pain, seizures fever,
pleurisy, Arthritis and rashes on the skin).4. Senile amyloidosis.
5. Dialysis amyloidosis develops during hemodialysis.
Causes
Causes of primary lesion of various organs (kidneys, intestines, skin) are unknown.
Symptoms and course of the disease are varied and depend on the location of amyloid deposits, the degree of their prevalence in the organs, duration of disease, presence of complications.
Often present with complex symptoms associated with damage to several organs.
Manifestations of amyloidosis:
*gastrointestinal tract
- Increasing the size of the language
- swallowing disorder
- Diarrhea or constipation
- Tumor-amyloid deposits in the stomach or intestines (not often)
esophageal amyloidosis is usually accompanied by lesions of other parts of the digestive system. Characterized by difficulty swallowing if swallowed dense and dry food, regurgitation.
Amyloidosis of the stomach is usually combined with amyloidosis of the intestine and other organs. Manifestations: a feeling of heaviness in the epigastric region after eating, heartburn, belching, nausea.
bowel Amyloidosis occurs frequently manifested a sense of discomfort, heaviness, less moderate abdominal pain, stool disorders (constipation or persistent diarrhea). Isolated tumor intestinal amyloidosis occurs under the guise of the tumor (pain, ileus), And is usually detected during surgery.
liver amyloidosis observed relatively often characterized by an increase in liver and seal often appear syndrome portal hypertensionRarely there is pain in the right upper quadrant, nausea, belching, jaundiceHemorrhage.
Amyloidosis of the pancreas usually takes place under the guise of chronic pancreatitis; characterized by a dull ache in the left upper quadrant, belching, nausea, vomiting.
* heart
- Treatment-resistant congestive heart failure
- Rhythm and conduction disturbances
- Focal lesions of the myocardium (psevdoinfarkt).
* Nervous System
- Peripheral polyneuropathy (burning sensation, tingling, "pins and needles" in the extremities, sensory impairment)
- Violations of the autonomic nervous system (headache, dizziness, sweating)
- orthostatic hypotension
- Impotence
- Sphincter disorders (urinary incontinence, fecal).
* Tendons, cartilages
- Carpal tunnel syndrome (numbness, tingling and throbbing pain in fingers)
- Symmetric polyarthritis, frozen shoulder, tight periarticular swelling.
* fabrics
- Enlargement of the spleen.
* Respiratory
- hoarseness
- Bronchitis
- Tumor pulmonary amyloidosis.
skin lesions - papules, plaques, nodes, bleeding around the eyes ( "symptom points").
As well as:
- The involvement of the thyroid gland and reduce its function.
- Involvement of the adrenal glands to their failure.
- Renal amyloidosis, typical for all forms of amyloidosis.
Diagnostics
- In the blood, anemia, leukocytosis, increased erythrocyte sedimentation rate, nearly 80% of cases of the disease occur in early reduction in the amount of proteins, hyperglobulinemia, reduction of platelet count, Sodium, calcium.
- When liver disease - raising cholesterol, in some cases - bilirubin, Increased alkaline phosphatase
- Assessment of thyroid function - may decrease thyroid function
- Assessment of renal function - almost 50% of cases begins amyloidosis with renal failure. In the study of urine, in addition to squirrelIn sediment show cylinders erythrocytes, leukocytes
- In primary amyloidosis in the blood plasma and / or urine detected increase of amyloid
- In secondary amyloidosis should pay attention to laboratory signs of chronic inflammatory diseases
- The feces - large amounts of fat, starch, muscle fibers
diagnostic measures
- Echocardiography (for suspected heart failure)
- X-ray inspection
- Functional clinical trials with Congo red and methylene blue (dye disappearance rapid intravenous administration of the serum due to their
- amyloid fixation and a significant reduction in their release by the kidneys). In primary amyloidosis, these tests are not always informative
- Biopsy of organs - the most informative method.
amyloidosis treatment
General principles of treatment
home mode, except for the severe conditions (severe heart failure, chronic renal failure)
In primary amyloidosis, in the initial stages of the process - chloroquine 0.25 g 1 r / d.. long, the combination of melphalan and prednisolone, melphalan, prednisone and colchicine colchicine or only
In secondary amyloidosis - treatment of the underlying disease (tuberculosis, osteomyelitis, Empyema et al.), After which curing symptoms often vanish and amyloidosis
Transfer the patient to the dialysis amyloidosis on peritoneal dialysis
When amyloidosis bowel flowing with persistent diarrhea - astringents (bismuth subnitrate, adsorbents)
In secondary amyloidosis - specific treatment of the underlying disease
Amyloidosis in familial - colchicine (0.6 mg 2-3 p / d.).
Symptomatic therapy: vitamins, diuretics, drugs that reduce pressure, plasma transfusions, etc...
Advice on nutrition
Patients amyloidosis shows a long-term (1.5-2 years) receiving raw liver (100-120 g / day).
The restriction of protein intake, salts of patients with chronic renal failure
salt restriction for patients with heart failure
Surgical treatment of amyloidosis
Removal of the spleen can improve due to reduced amount of amyloid formed in the body.
Forecast
The prognosis of amyloidosis is determined by the underlying disease. The average survival rate - 12-14 months. Women often live longer. The disease is more severe in the elderly.