In medical terminology, Goodpasture Syndrome means a special syndrome that is determined by autoimmune disorders leading to damage to the basement membranes of the alveoli of the lungs, as well as renal glomeruli, i.e., two organs are involved in the pathological process: lungs and kidneys. Antibodies to these organs are formed in the body.
All of the above synonymous syndromes are aggravated by nephritis and glomerulonephritis.
The key manifestation is recurrent and progressive pulmonary hemorrhage in combination with glomerulonephritis.
We will understand what kind of disease is Goodpasture syndrome.
History and Statistics
The clinical manifestations of this disease were first described and systematized by Goodpascher in 1919, hence the name of this syndrome. During a flu pandemic, this syndrome was isolated into a separate burdened syndrome, such as a combination of glomerulonephritis and pulmonary hemorrhage.
This is a rather rare pathology - Goodpasture syndrome and hemoptysis, they are more often affected by young people from 12 to 35 years old, mostly males.
In European countries, in which it is relatively more common, 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 are the following possible causes of Goodpasture syndrome:
- Chemical tissue damage resulting from aspiration of organic solvents and volatile hydrocarbons.
- Some researchers consider this syndrome not as a separate disease, but as a variation of idiopathic hemosiderosis of the lungs. This assumption is based on indirect evidence of a transition state between these two diseases, both clinically determined and pathomorphologically.
- The procedure for crushing stones with urolithiasis.
- Genetic nature, some HLA genes.
- They also have assumptions about the viral origin of the disease, but there are not enough facts collected regarding this assumption.
The pathogenesis of Goodpast syndrome
Let us consider in more detail the anatomical features of the structure of the affected organs and the features of this pathology.
With Goodpasture disease, damage to the alveoli and glomeruli of the kidneys occurs.
Alveoli are cluster-shaped respiratory vesicles that are located at the ends of the smallest bronchioles. The walls of the alveoli have two layers: the epithelial layer, which has direct contact with air, and the layer of endothelial cells, which are located on the walls of blood capillaries. The gap between the said layers contains a special membrane, a basement membrane, into which oxygen and carbon dioxide penetrate.
Renal glomeruli are the building blocks of the kidneys, their smallest functional unit. They consist of a capsule and a capillary network, which is located in this capsule. The inner surface of the capillaries contains a special layer of the endothelium, and the outer side facing the capsule is represented by podocytes. They are separated by a basement membrane, which has a throughput function - it passes salts, water, and proteins from the blood into the capsule. The location of the basement membrane is not limited to the above arrangement. She also shares the renal tubules, whose function is to remove primary urine. It also divides 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 ensures the removal of carbon dioxide and substances formed during their exchange in the body, and also provides oxygen.
Accordingly, if the membrane is damaged, all these metabolic processes are violated.
Antibody formation
Above in the definition, we noted that damage to the membrane tissue is due to the formation of antibodies to it itself (protective substances). 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 formed antibodies to penetrate into it, nevertheless, in the presence of certain unfavorable conditions, vascular permeability increases, as a result of which the antibodies nevertheless penetrate the basement membrane.
Unfavorable factors
These adverse factors for Goodpast syndrome include:
- increased oxygen concentrations in the inhaled air;
- pulmonary hypertension;
- septic lesions of the blood 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 primarily affected, the kidneys or the lungs, has not been established. However, the incidence of primary lung tissue lesions is higher than that of renal tissue.
Histological tests of the lung tissue show the presence of necrotizing alveolitis. As we noted above, this is a pathological tissue change similar to that of idiopathic hemosiderosis.
Histological features of pathology
Timely diagnosis of Goodpasture syndrome is very important.
Histological samples of renal tissue detect the presence of nephrosiephritis (a combination of lipoid degenerative 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 syndrome?
Symptoms and laboratory indicators
These include:
- hemoptysis and anemia as a result of recurrent pulmonary hemorrhage;
- progressive symptoms in the form of shortness of breath, weight loss, pain in the chest;
- X-ray of the lungs shows changes in individual small foci of the pulmonary structure in the form of a fine-mesh deformation;
- symptoms of kidney damage: as we noted above, they often join after damage to the lung tissue;
- a urinalysis reveals the presence of protein, and blood is also detected in the urine.
- a blood test reveals an increased level of residual nitrogen, progressive hypochromic iron deficiency anemia, against the background of the secondary infection, the blood formula reflects this.
It should be noted that pulmonary hemorrhage 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 literally in a matter of hours. Severe shortness of breath develops, pulmonary edema occurs and fulminant pneumonia develops.
Clinical picture
Against the background of the general characteristics of the above symptoms, it is customary to distinguish three forms of the course of this systemic disease:
- Malignant form. It is characterized by the presence of the recurrent nature of pneumonia and the rapid development of glomerulonephritis.
- Slow development of pathological changes in the pulmonary and renal tissues.
- Glomerulonephritis, which progresses and prevails over pulmonary changes, leads to acute renal failure in the shortest possible time.
In children
Why does pathology occur in a child? The reason for this is the hereditary predisposition of the child, which can be infected from their parents. In most cases, the disease develops in babies whose mothers led an unhealthy lifestyle and smoked during the period of bearing the child. As a result, the baby in the development process did not receive the oxygen necessary for the body, and its lungs simply turned into the lungs of a smoker. In addition, additional factors can negatively affect the baby’s health, such as, for example, the presence of viral infections affecting the respiratory system, as well as inhalation of hydrocarbon vapors.
Goodpasture 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 sonorous wet wheezing 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 systemic disease 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 a clear airway is a rather important point.
Given that Goodpasture Syndrome is developing quite actively in both young and adult body, in the absence of a correctly conducted diagnosis and effective treatment, the disease can lead to more complex consequences. Moreover, in medical practice there have been cases of death.
Forecasts
The prognosis of the pathology, unfortunately, is unfavorable. A patient dies on average during the year. As we noted above, there are also forms of 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.
The mechanism of the development of the disease
Goodpasture syndrome is an autoimmune disease, that is, caused by the generation of antibodies to own cells. The renal glomerular basement membrane is the site where these antibodies form. These antibodies bind to a specific domain in the fourth type of collagen.
It is this part of collagen of the fourth type that is the target point of antibodies. This part of collagen of the fourth type is called Goodpasture antigen.
In healthy people, this antigen is not a trigger for pathological chains. The disease affects the lung and kidney tissue, because it is in these tissues that a large number of antigens of this type are contained.
With the formation of the association of antibodies with Goodpastor antigen, the complement system is launched. These are immune proteins, or rather their special form. This formed bond is the trigger of the pathological chain protein reaction. In the area of contact of antibodies and antigens, a connection with white blood cells occurs.
Everything goes to the fact that white blood cells actively attack the affected areas of the tissue, thereby destroying them. The immune response to this process is a sharp increase in the number of epithelial cells. Significant deposition occurs on the surface of the basement membrane. The body begins to quickly lose its functionality, can not cope with the load, vital products quickly accumulate in the body.
The mechanisms of pathological changes in the kidneys and lungs have an identical course.
Treating Goodpasture Syndrome
There are only methods to slow the progression of the disease in order to prolong the patient's life. To this end, corticosteroids and immunosuppressants are prescribed, hemodialysis is performed, after which nephrectomy and kidney transplantation are performed, if necessary, which eliminates the source of antigenic reactions. Plasmapheresis removes circulating antibodies.
It was noted that antibodies against basement membranes are not detected in serum on average six months after the onset of the disease. That is why there is an assumption that the aforementioned intensive care measures can extend the patient's life until the pathological autoimmune process stops.
Symptomatic measures include blood transfusion and the fight against iron deficiency anemia.
With Goodpasture's Syndrome, clinical guidelines should be strictly followed.