Treatment
The basis of the treatment of Goodpasture’s syndrome is the administration of immunosuppressive drugs (such as cyclophosphamide) and glucocorticoids (i.e. cortisone) and a plasma exchange (“plasmapheresis”) to remove the circulating antibodies.The survival after 1 year in a British retrospective is 100% and the survival of the kidneys is 95%. According to recent findings, an effective supplement of the treatment consists of antibodies, which 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 long-term prognosis.
Frequency
Goodpasture’s syndrome, like all autoimmune diseases, is a very rare disease. It is estimated that for every 1 million inhabitants, there is 1 person suffering from Goodpasture’s syndrome. Men seem to be affected somewhat more frequently than women.
The age of the patients is very variable, cases from 10 to 90 years are reported. The disease peaks, i.e. the age at which most patients develop the disease, are around 30 and around 60 years of age (60-year-olds are often women who have already been diagnosed with kidney disease once in their lives). According to a Chinese study on Goodpasture’s syndrome, the older patients usually have a somewhat milder course of Goodpasture’s syndrome.
Course of the disease
At the beginning of the disease, there are hardly any or very few autoantibodies present and therefore the symptoms are rather subtle. A slight microhaematuria can already be detected in the urine test (i.e. there is already blood in the urine, but in such low concentrations that it would not be visible to the naked eye at all, but can only be diagnosed with the help of special urine test strips). The incipient damage to the kidneys can lead to renal hypertension, high blood pressure caused by reduced kidney function, which in turn can cause symptoms and complications.
In most cases, however, a detailed diagnosis is not initiated until Goodpasture’s syndrome is already at an advanced stage and the patient is suffering from coughing up blood and the blood in the urine is also clearly visible to the human eye. These two characteristics – the coughing up of blood and the blood in the urine – should prompt an experienced physician to consider Goodpasture’s syndrome, especially if the kidney failure is progressing rapidly. Renal biopsy (the collection of small samples from the kidney in a minor surgical procedure) is the safest way to diagnose Goodpasture’s syndrome.
The kidney sample sent to the histology laboratory can then be used to diagnose rapid progressive glomerulonephritis. In direct immunofluorescence (another examination option for physicians in a histological laboratory) with fluorescent antibodies against human immunoglobulin, a linear fluorescence is found on the glomerular basement membrane (quasi the “baseplate” of the kidney tissue). In rare cases, rapidly progressing renal insufficiency dominates without a coughing up of blood.
Among the laboratory values analyzed from blood samples, kidney values increase rapidly and strongly and circulating anti-GBM antibodies can also be detected. The severity of the symptoms the patient suffers from depends on the amount of antibodies produced in the body. Once the coughing up of blood begins, an advanced stage of Goodpasture’s syndrome has already been reached and immediate diagnosis and therapy is of utmost importance.
If the course of the disease is foudroiant (= very stormy), respiratory insufficiency and renal insufficiency also occur within a short time. Due to the blood loss via urine, patients develop severe anemia (a lack of blood). As the kidneys become more and more affected, hypertension and renal insufficiency become more pronounced.
In the past, a disease like Goodpasture’s syndrome was always fatal. Today, however, thanks to the modern methods and possibilities of modern medicine, it is possible to treat at least the lung involvement well. However, once the kidneys have become insufficient, they cannot be healed again, so that a renal replacement therapy, i.e. dialysis, or even a kidney transplantation must be considered.
