Goodpasture's Syndrome - Causes, Symptoms and Treatment .MF .
Goodpasture's syndrome - a disease that occurs when there is a defect of the immune system and is characterized by pulmonary hemorrhage and rapidly progressive glomerulonephritis (inflammation of the kidney tissue).
frequency of occurrence of the disease is 1 case per 2,000,000 population.The first peak incidence between the ages of 20-30 years, and predominantly male ill, the second peak age 50-60 years, both sexes suffer from the same frequency.
Causes Goodpasture syndrome
reason Goodpasture's syndrome is unknown.There are indications that the disease is associated with a viral infection particularly influenza virus.Some environmental factors, such as organic solvents, gasoline, some drugs may be predisposing factors of the disease.It is known that pulmonary hemorrhage occurred, usually in smokers, that is, prior lung damage also plays a major role in causing the disease.
In Goodpasture's syndrome is based on the formation of specific proteins - antibodies specific for vascular cells.Under the in
symptoms of Goodpasture syndrome
to onset of the disease characterized by the development of non-specific symptoms in the form of weakness, drowsiness, fever up to 37-37,5 degrees, weight loss.Sometimes the first sign of the disease is a low concentration of hemoglobin in the absence of any bleeding.
for Goodpasture's syndrome is characterized by lung and kidney.
• Lung disease usually first appears hemoptysis (blood in sputum appearance of veins, as well as individual spitting liquid or partially clotted blood in expectoration).
This symptom is usually several months ahead of kidney disease.Hemoptysis may be constant or occur periodically.In addition, disturb the patient pain in the chest, shortness of breath at rest or with exercise, coughing.
Especially dangerous is the development of pulmonary hemorrhage.This condition is characterized by momentary expectoration of more than 100 ml of liquid blood.With the defeat of the great vessels, bleeding may be potentially life-threatening.Even a slight hemoptysis, not to mention the bleeding leads to a decrease in hemoglobin and development of iron deficiency anemia.Very often, lung disease complicated by pneumonia (inflammation of the lungs) or pulmonary edema (fluid accumulation in the lung tissue above the physiological norm, is characterized by symptoms of respiratory failure).
• If kidney damage develops glomerulonephritis.At the onset of the disease a moderate amount of protein and red blood cells appear in the urine.Massive proteinuria or edema syndrome do not have time to develop, because it is a feature of the disease rapidly progressive character.Within a few weeks of sharply reducing the amount of urine and formed terminal renal failure with severe renal impairment.
Goodpasture syndrome Diagnostics
Goodpasture Syndrome Diagnosis is based on detection of antibodies to the cells of blood vessels by ELISA.The general analysis of blood noteworthy decrease in hemoglobin concentration as a result of blood loss, increased white blood cell count and erythrocyte sedimentation rate accelerated, reflecting the presence of inflammation.If it affects the lungs on radiographs determined infiltration foci.This tissue sections from lung hemorrhage.
Treatment Goodpasture syndrome
Treatment Goodpasture's syndrome should be timely and intense.Used hormones (prednisolone) and cytotoxic drugs (cyclophosphamide).With the aim of replacement transfusions of packed red cells and plasma.With the development of end-stage renal failure, dialysis sessions are held.
Unfortunately, approximately 40% of the cases the treatment is ineffective, and patients die of pulmonary hemorrhage, or from poisoning by toxic substances in violation of renal excretory function.Currently, transplantation (transplantation) the kidneys are not helping to reduce mortality.
physician therapist, nephrologist Sirotkin EV