It is a rare but well-described disease that presents with rapidly progressive glomerulonephritis and pulmonary haemorrhage.
The disease was first described in 1919 by the American physician and pathomorphologist Ernest, William Godpasture, and its name derives from his surname. The disease is included in the group of autoimmune diseases because the body itself produces antibodies against the basement membrane of the glomeruli.
The exact number of people suffering from this syndrome is not known as there is no precise medical data. Estimates show that in Europe, 0,5-1 people per 100 people suffer from this disease. residents. It is known, however, that the disease affects men almost 3 times more often than women.
Course of illness
When the disease develops, acute renal failure, nephrotic syndrome and pathological changes in the glomeruli may occur. Blood spitting may occur on the part of the respiratory system as a result of bleeding from the alveoli in the lungs. The disease is rarely accompanied by a general malaise, weakness or lack of appetite.
Kidney involvement almost always occurs. Pulmonary symptoms in young people occur in 70-80 percent. cases, while in patients over 60 years of age much less often, i.e. in 20% of patients. The prognosis for patients with Goodpasture’s syndrome is also difficult to predict, because the success of therapy depends on the degree of kidney and lung damage.
The clinical symptoms of the disease are caused by a type II hypersensitivity reaction, leading to the production of antibodies against the basement membrane of the glomeruli, and more specifically against type IV collagen. Type IV collagen is found in the basement membrane of the glomeruli and alveoli. In about 85 percent. In cases of Goodpasture’s syndrome, the antigen is the non-collagen NC1 domain of the 3 alpha chain of type IV collagen. These antibodies most often belong to the IgG class, present both in the blood and in situ in the tissue of the kidneys and lungs.
The diagnosis of Goodpasture’s syndrome requires the presence of clinical symptoms and anti-GBM antibodies detected and confirmed by special tests.
Goodpasture’s syndrome is a severe autoimmune disease with rapidly progressive glomerulonephritis and pulmonary haemorrhage, which, along with infections, is the most common cause of death. The diagnosis of this syndrome is based on the detection of anti-GBM antibodies and performing a kidney biopsy with examination in an immunofluorescence microscope.
Patients require complex treatment consisting of glucocorticosteroids, cyclophosphamide and plasmapheresis. Many people require dialysis, which extends their life significantly. If treatment is started early, there is usually no permanent damage to the lungs. Often, however, the kidneys are so malfunctioning that constant renal replacement therapy is necessary.