The Goodpasture Syndrome, in English anti-glomerular basement membrane (GBM) disease / anti-GBM disease, is one of numerous violent, but fortunately only rarely occurring Autoimmune diseases. In autoimmune diseases, your own body forms antibody, actually "good antibodies" of our body's own immune system, against the body's own structures or cells. These antibodies are normally only formed after a person has come into contact with a substance that is foreign to the body, has recognized it as such thanks to its unknown surface structures and then activates the body's own defense cells. These are then formed and shaped specifically for this one type of foreign substance and thus potential pathogens. So you can only recognize this type and are otherwise harmless, so no further harm to our body. But if it happens that the body suddenly or gradually no longer recognizes familiar body structures as belonging to it, it also classifies them as foreign bodies and begins to treat them as such. What follows is also the actually quite normal cascade-like one that is necessary for survival Defense reaction of the body - only now the body's own structures are attacked and fought.
Causes of Goodpasture Syndrome
The development of an autoimmune disease can have a wide variety of causes. For example there are certain genetically determined and hereditary autoimmune diseases. Others are acquired, for example through an earlier one Infection with bacteria or viruses. Others can get through too toxic substancesthat one has come into contact with. Last but not least, in a large number of cases it is easy not clear and no further trigger can be determined.
The causes of Goodpasture Syndrome are completely unclear, but it is believed that there are a number of triggering factors that work together. Apparently they're playing Pre-existing illness the patient plays an important role, for example the flu (as was the case with the patient who was first described in 1919 by the American pathologist Ernest Goodpasture (October 17, 1886 to September 20, 1960), here too it was the one previous one Influenza infection). Links to pulmonary tuberculosis were also described. In Goodpasture's syndrome, an allergic-hyperergic type 2 reaction Autoantibodies (mostly IgG1 and IgG4, but in 1/3 of the cases also IgA and IgM) specifically against the Type IV collagen in the basement membranes the glomeruli (the smallest units of the kidney called kidney corpuscles), which are also found in the lungs. This explains why the same autoantibodies destroy the basement membranes of both the lungs and the kidneys. In the lungs it leads to Bleeding into the lung tissue even in and to bloody cough. In the kidneys, blood passes into the (primary) urine and hematuria occurs.
In Goodpasture's syndrome, the body begins to form antibodies against the basement membrane (which are therefore called anti-GBM antibodies), which then ultimately becomes one severe damage to the kidneys and lungs leads.Due to kidney involvement, it can happen sooner or later that the person affected develops hematuria, i.e. blood can be found in the urine, and suffers from hypertension (since the kidney is an essential part of the human blood circulation regulation system).
In Goodpasture's syndrome, the body begins to form antibodies against the basement membrane (which are therefore referred to as anti-GBM antibodies), which ultimately leads to severe damage to the kidneys and lungs. Due to kidney involvement, it can happen sooner or later that the person affected develops hematuria, i.e. blood can be found in the urine, and suffers from hypertension (since the kidney is an essential part of the human blood circulation regulation system).
Goodpasture's syndrome is diagnosed via the laboratory detection of anti-basement membrane antibodies and immunoglobulin deposits on basement membranes of the glomerula, which are taken in a kidney biopsy from one or both kidneys.
The basis of the treatment of Goodpasture's syndrome is the administration of Immunosuppressants, (such as Cyclophosphamide) and Glucocorticoids (i.e. cortisone) and a plasma exchange ("Plasmapheresis“) To remove the circulating antibodies. Survival at 1 year in a UK retrospective is 100% and kidney survival is 95%.
According to recent findings, an effective addition to the treatment is Antibodiesthat intervene in the immunological process. The antibody Rituximab (MabThera®) against a surface antigen of B lymphocytes and pre-B lymphocytes apparently leads to a significant improvement in the long-term prognosis.
Goodpasture syndrome, like all autoimmune diseases, is one very rare disease. It is estimated that to around 1 million inhabitants 1 goodpasture syndrome sufferer. Men seem to be affected slightly more often than women. The age of the patients is very variable, cases from 10 to 90 years have been reported. The Illness peak, the age at which most patients got it at around 30 and around 60 years (The 60-year-olds are often women who have had kidney disease at one point in their life). According to a Chinese study on Goodpasture syndrome, the older patients mostly have a somewhat milder course of Goodpasture syndrome.
Course of the disease
At the beginning of the disease there are hardly any or very few autoantibodies present and therefore the Symptoms also rather subtle. In the urine test you can already see a slight Microhematuria determine (i.e. that there is already blood in the urine, albeit in such low concentrations that it cannot be seen with the naked eye, but can only be diagnosed with the help of special urine test strips). The incipient kidney damage can lead to a renal hypertension come, one high blood pressure caused by impaired kidney functionwhich in turn can cause symptoms and complications. In most cases, however, a detailed diagnosis is only initiated when the Goodpasture syndrome is already at an advanced stage and the patient has responded Coughing up blood suffers and that Blood in the urine is also easily visible to the human eye is.
These two characteristics - coughing up blood and blood in the urine - should also make an experienced doctor think of Goodpasture syndrome immediately, especially if kidney failure is rapidly advancing.
The Kidney biopsy (the taking of small samples from the kidney in a small surgical procedure) is the safest way to diagnose Goodpasture syndrome. Rapid progressive glomerulonephritis can then be detected in the kidney sample sent to the histological laboratory. In the direct immunofluorescence (Another possibility for doctors to examine in a histological laboratory) with fluorescent antibodies against human immunoglobulin, linear fluorescence is found on the glomerular basement membrane (the "baseplate" of the kidney tissue). In rare cases, rapidly progressing renal insufficiency dominates without coughing up blood. Among the laboratory values analyzed from the blood sample, the kidney values rise rapidly and sharply and one can also see circulating ones Anti-GBM antibodies prove. The severity of the symptoms the patient is experiencing depends on the amount of antibodies made in the body. If the coughing up of blood sets in, an advanced stage of Goodpasture's syndrome has already been reached and immediate diagnosis and initiation of therapy is of the utmost importance. In the case of a foudrant (= very stormy) course of the disease, another occurs within a short time Breathing insufficiency and kidney failure. Due to the loss of blood from the urine, patients develop severe anemia (a Anemia). As the kidneys are more and more affected, the hypertension and renal insufficiency become more pronounced.
In the past, a disease like Goodpasture Syndrome was always fatal. Today, however, thanks to the modern methods and possibilities of today's medicine, at least lung involvement is possible treat well to be able to. However, once the kidneys have become insufficient, they cannot heal again, so that a Renal replacement therapy, i.e. dialysis, or a kidney transplant must be considered.