In medical terminology, Goodpasture's syndrome means a special syndrome, which is determined by autoimmune disorders leading to damage to the basement membranes of the alveoli of the lungs, as well as to the renal glomeruli, i.e. two organs are involved in the pathological process: lungs and kidneys. The body produces antibodies to the listed organs.
All of the above synonymous syndromes are aggravated by nephritis and glomerulonephritis
The key manifestation is recurrent and progressive pulmonary bleeding in combination with glomerulonephritis.
Let's figure out what kind of disease Goodpasture's syndrome is.
History and statistics
The first clinical manifestations of this disease were described and systematized by Goodpasture in 1919, hence the name of this syndrome. During the influenza pandemic, this syndrome was isolated as a separate aggravated syndrome, such as a combination of glomerulonephritis and pulmonary hemorrhage.
This is a fairly rare pathology - a syndromeGoodpasture and hemoptysis, they are more common in young people from 12 to 35 years old, mostly male.
In European countries, where it occurs relatively more often, the incidence is 1:2 million.
Etiology of the syndrome
In modern medicine there is no single answer regarding the etiology of the disease
There are only a few suggestions, among which the following possible causes of Goodpasture's syndrome can be distinguished:
- Chemical damage to tissue as a result of aspiration of organic solvents and volatile hydrocarbons.
- Some researchers consider this syndrome not as a separate disease, but as a variation of idiopathic pulmonary hemosiderosis. This assumption is based on circumstantial evidence for the existence of a transitional state between the two diseases, both clinically and pathologically determined.
- The procedure for crushing stones in urolithiasis.
- Genetic nature, some HLA genes.
- Also have speculations about a viral origin of the disease, but not enough facts have been collected regarding this speculation.
Pathogenesis of Goodpasture's syndrome
Let's consider in more detail the anatomical features of the structure of the affected organs and the features of this pathology.
With Goodpasture's disease, the alveoli and glomeruli of the kidneys are affected.
Alveoli are cluster-shaped respiratory vesicles that are located at the ends of the smallestbronchioles. The walls of the alveoli have two layers: a layer of epithelium, which has direct contact with air, and a layer of endothelial cells, which are located on the walls of blood capillaries. The gap between these layers contains a special membrane, the basement membrane, into which oxygen and carbon dioxide penetrate.
Renal glomeruli are the building material of the kidneys, their smallest functional unit. They consist of a capsule and a capillary network that is in this capsule. The inner surface of the capillaries contains a special layer of endothelium, and the outer side, facing the capsule, is represented by podocytes. Between themselves, they are separated by a basement membrane, which has a throughput function - it passes s alts, water, proteins from the blood into the capsule. The location of the basement membrane is not limited to the location described above. It also separates the renal tubules, whose function is to excrete primary urine. It also separates the blood capillaries into which fluid is absorbed from it.
Summarizing all of the above, it can be noted that the basement membrane is a kind of biological filter that removes carbon dioxide and substances formed during their metabolism in the body, and also provides oxygen.
Accordingly, if the membrane is damaged, all these metabolic processes are disrupted.
Antibody formation
Above in the definition, we noted that damage to the membrane tissue is due to the formation of antibodies to itself (protectivesubstances). This process belongs to the category of autoimmune. It turns out that antibodies attack their own tissue, forming deposits of the pathological layer, thereby damaging it. The result of this autoimmune process is pulmonary hemorrhage and glomerulonephritis - an inflammatory process of the renal glomeruli.
Although the structure of the vascular endothelium of the pulmonary capillaries is such that it does not allow the resulting antibodies to penetrate into it, nevertheless, under certain unfavorable conditions, an increase in vascular permeability occurs, as a result of which antibodies still penetrate to the basement membrane.
Unfavorable factors
Such adverse factors of Goodpasture's syndrome include:
- increased concentrations of oxygen in the inhaled air;
- pulmonary hypertension;
- septic blood lesions or general intoxication of the body;
- infectious processes of the upper respiratory tract;
- smoking;
- aspiration of gasoline vapors or other types of hydrocarbon derivatives.
What is affected primarily, the kidneys or lungs, has not been established. However, the frequency of manifestations of the primary lesion of the lung tissue is higher than that of the renal tissue.
Histological tests of lung tissue show the presence of necrotizing alveolitis. As we noted above, this is a pathological tissue change similar to that in idiopathic hemosiderosis.
Histological features of pathology
Timely diagnosis of the syndromeGoodpasture is very important.
Histological tests of the kidney tissue reveal the presence of nephronephritis (a combination of lipoid dystrophic degeneration of the kidneys and diffuse glomerulonephritis). The development of focal intracapillary thrombotic changes and glomerular fibrosis is also detected.
What are the symptoms of Goodpasture's syndrome?
Symptomatology and laboratory findings
These include:
- hemoptysis and anemia as a result of recurrent pulmonary bleeding;
- progressive symptoms of shortness of breath, weight loss, chest pain;
- X-ray of the lungs shows changes in individual small foci of the lung structure in the form of a fine-mesh deformity;
- symptoms of kidney damage: as we noted above, they often join after damage to the lung tissue;
- urinalysis detects the presence of protein, blood is also detected in the urine.
- blood test reveals elevated levels of residual nitrogen, progressive hypochromic iron deficiency anemia, against the background of secondary infection, the blood count reflects that.
It is worth noting that pulmonary bleeding does not always occur against the background of hemoptysis, and the severity of bleeding does not depend on the intensity of hemoptysis. Very often, bleeding occurs at lightning speed and leads to a fatal outcome in just a matter of hours. Severe shortness of breath develops, pulmonary edema occurs, and fulminant pneumonia develops.
Clinicalpicture
Against the background of the general characteristics of the symptoms described above, it is customary to distinguish three forms of the course of this systemic disease:
- Malignant form. It is characterized by the presence of a recurrent nature of pneumonia and the rapid development of glomerulonephritis.
- Slow development of pathological changes in the lung and kidney tissues.
- Progressive glomerulonephritis that prevails over pulmonary changes in the shortest possible time leads to acute renal failure.
In children
Why does a child develop pathology? The reason for this is the hereditary predisposition of the child, who can be infected from his parents. In most cases, the disease develops in babies whose mothers during the period of bearing a child led an unhe althy lifestyle and smoked. As a result, the baby in the process of development did not receive the oxygen necessary for the body, and his lungs simply turned into the lungs of a smoker. In addition, additional factors can negatively affect the he alth of the baby, such as, for example, the presence of viral infections that affect the respiratory system, as well as the inhalation of hydrocarbon vapors.
Goodpasture's syndrome in children often begins very acutely, with a rise in temperature to high values, hemoptysis, pulmonary hemorrhage, intense shortness of breath appear. Listening reveals voiced moist rales in the lungs. Glomerulonephritis develops most often, though later, but quickly enough. As noted above, the development of renal failure follows almost instantly. A characteristic immunological marker of systemicdisease is the presence of antibodies to the basement membranes of the kidney.
It is necessary to treat such diseases, especially in children, under the strict supervision of specialists, while ensuring airway patency is quite important.
Given that Goodpasture's syndrome develops quite actively both in young and adult organisms, in the absence of a properly performed diagnosis, as well as effective treatment, the disease can lead to more complex consequences. Moreover, there have been deaths in medical practice.
Forecasts
The prognosis of pathology, unfortunately, is unfavorable. The patient dies on average within a year. As we noted above, there are also forms of a fulminant course of the disease, when literally a week passes from the first signs of the disease in the form of fever to a fatal outcome.
Mechanism of disease progression
Goodpasture's syndrome is an autoimmune disease, that is, caused by the generation of antibodies to one's own cells. The glomerular basement membrane is where these antibodies are made. These antibodies bind to a specific domain in the fourth type of collagen.
It is this part of the fourth type of collagen that is the target point of antibodies. This part of collagen type 4 is called the Goodpasture antigen.
In he althy people, this antigen is not the trigger of pathological chains. The disease affects the lung and kidney tissue, because it is in these tissues thata large number of antigens of this species.
When an antibody binds to the Goodpasture antigen, the complement system is triggered. These are immune proteins, or rather their special kind. This bond formed is the trigger mechanism for a pathological protein chain reaction. In the focus of contact between antibodies and antigens, a connection occurs with leukocytes.
Everything goes to the fact that leukocytes actively attack the affected tissue, thereby destroying them. The immune response to this process is a sharp increase in the number of epithelial cells. Their significant deposition occurs on the surface of the basement membrane. The organ begins to quickly lose its functionality, cannot cope with the load, waste products quickly accumulate in the body.
Mechanisms of pathological changes in the kidneys and lungs have an identical course.
Treatment of Goodpasture's syndrome
There are only methods to slow down the progression of the disease in order to prolong the life of the patient. For this purpose, corticosteroids and immunosuppressants are prescribed, hemodialysis is performed, after which, if necessary, nephrectomy and kidney transplantation are performed, which allow eliminating the source of antigenic reactions. Plasmapheresis removes circulating antibodies.
It was noticed that antibodies against basement membranes are not detected in the blood serum, on average, six months after the onset of the disease. That is why there is an assumption that the above measures of intensive care can extend the life of the patient up totermination of the pathological autoimmune process.
Symptomatic measures include blood transfusions and iron deficiency anemia.
In Goodpasture's syndrome, clinical guidelines must be strictly followed.
