Rapidly progressive glomerulonephritis (crescentic glomerulonephritis) is a type of glomerulonephritis. It is characterized by its rapidly progressive course.
What is rapidly progressive glomerulonephritis?
Glomerulonephritis is an abacterial inflammation that usually affects both kidneys. Rapidly progressive glomerulonephritis (RPGN) presents with the typical symptoms of kidney inflammation. However, the symptoms in this form worsen very rapidly, so that kidney failure can occur within a very short time. Rapidly progressive glomerulonephritis is therefore an emergency that requires rapid intensive medical treatment. Based on its causes, rapidly progressive glomerulonephritis can be divided into three types.
Causes
Type 1 of RPGN is caused by antibodies against the membrane of the renal corpuscles. An example of this form of glomerulonephritis is Goodpasture’s syndrome. Here, the cell walls of the kidney and lung express the so-called Goodpasture antigen. Antibodies bind to this antigen, causing severe inflammation. About twelve percent of all rapidly progressive glomerulonephritis is caused by antibodies. More commonly, rapidly progressive glomerulonephritis results from the deposition of immune complexes. An immune complex is a complex of an antibody and an antigen. It is formed by an antigen-antibody reaction. Most often, these antigen-antibody complexes develop during infections. They circulate in the blood and then deposit on the basement membrane of the renal corpuscles. There they cause an immune reaction with subsequent inflammation. This type also frequently develops in the context of autoimmune diseases. One example of such an autoimmune disease with kidney involvement is lupus erythematosus. Immune complexes and antibodies are absent in 44 percent of all patients with rapidly progressive glomerulonephritis. This type of RPGN is also called pauci-immune nephritis. The exact pathomechanisms are still unknown in this case. However, some patients suffer from autoimmune chronic vascular inflammation such as Wegener’s disease or microscopic polyangiitis. Inflammation of the glomeruli is possible at any age. Whereas postinfectious glomerulonephritis is more common in younger patients, rapidly progressive glomerulonephritis is typical in patients of older age.
Symptoms, complaints, and signs
Macrohematuria is characteristic of RPGN. In macrohematuria, blood is excreted in the urine. This is visible to the naked eye. Thus, the urine is reddish in color. Damage to the renal corpuscles leads to the so-called nephrotic syndrome. There is proteinuria. Patients therefore excrete increased amounts of protein in their urine. Due to the lack of protein, fluid accumulates in the tissues, causing edema. The edema occurs preferentially on the lower legs and eyelids. The body tries to compensate for the loss of protein by producing more hyperlipoproteins. Therefore, hyperlipoproteinemia develops with elevated triglyceride and cholesterol levels. The associated lipoproteins are also elevated. In the context of rapidly progressive glomerulonephritis, there is also an increase in blood pressure (hypertension). In contrast to normal glomerulonephritis, there is almost always an increase in retention values in rapidly progressive glomerulonephritis. Retention values are the renal values that reflect the functional capacity of the kidney. They include creatinine, creatinine clearance, urea, and cystatin C.
In RPGN, retention values increase rapidly as the kidney is severely damaged within a short period of time. Eventually, complete urinary retention occurs. Renal failure is imminent. In terminal renal failure, patients must undergo dialysis. Uremia develops with symptoms such as headache, vomiting, drowsiness, anemia or heart failure. In complicated courses of RPGN, pulmonary edema can also develop. In this case, fluid enters the alveoli. Affected patients then suffer from severe shortness of breath.
Diagnosis and course of the disease
If rapidly progressive glomerulonephritis is suspected, a urine status is first obtained. Blood cells and proteins are found in the urine.Serological methods are used to search for antinuclear factors such as ANF, ANCA, and for antibasal membrane antibodies. A kidney biopsy is performed to confirm the kidney. Cells are taken from the kidney and then examined histologically by a pathologist. This reveals necrosis and thrombosis of the glomeruli. The endothelial cells, podocytes, and mesangium are enlarged. In addition, crescent-shaped fibrinous foci can be found in the structure of the glomeruli. T cells and phagocytes can be detected within the resulting lesions. Patients in whom less than 80 percent of the glomeruli have crescent-shaped changes have better prognoses. A significant improvement in prognosis can also be achieved by early therapy. Without timely therapy, rapidly progressive glomerulonephritis almost always ends in chronic renal failure. In the worst cases, lifelong dialysis or kidney transplantation are necessary.
Complications
Rapidly progressive glomerulonephritis is a medical emergency that leads to severe renal failure if left untreated. It is already a complication of certain autoimmune diseases, the common feature of which is that the body’s immune system attacks kidney tissue. Often the disease is part of a systemic disorder. Therefore, other organs such as the lungs, among others, are often involved in the disease process. The most important complication of rapidly progressive glomerulonephritis is the rapid deterioration of kidney function. This can occur within days, weeks or months. If therapy is started too late, there is always a risk of needing dialysis or even complete kidney failure. Then a kidney transplant becomes necessary. The most severe course is the so-called Goodpasture syndrome, in which antibodies are formed against both the glomeruli and the pulmonary alveoli. Kidney and lung tissue are destroyed simultaneously. This form of rapidly progressive glomerulonephritis leads to death within a few days if left untreated. Goodpasture’s syndrome is characterized by a particularly rapid reduction in renal function, pulmonary infiltrates, and hemoptysis. Rapidly progressive glomerulonephritis may also cause severe hypertension and hyperkalemia. Death may occur from renal or pulmonary failure as well as from the effects of hypertension and hyperkalemia. Depending on the severity of hyperkalemia, paralysis, acidosis, intestinal obstruction, various central nervous disorders, and even severe cardiac arrhythmias with cardiac arrest or sudden cardiac death occur.
When should you go to the doctor?
A doctor should always be consulted for this type of glomerulonephritis. This disease cannot be treated by self-help means, so the affected person always needs medical treatment. As a rule, a doctor should be consulted when the affected person suffers from the clear signs of protein deficiency. This can lead to the formation of edema, which can affect the aesthetics of the affected person. Similarly, there may be a sharp rise in blood pressure, which may also be indicative of glomerulonephritis. In the worst case, the affected person may also suffer from renal insufficiency. Not infrequently, severe drowsiness or a lack of blood also point to this glomerulonephritis. If these complaints occur for a longer period of time, a doctor must be consulted. This can prevent further complaints of the heart, which can be fatal. The disease is treated by an internist. Under certain circumstances, glomerulonephritis also limits the life expectancy of the affected person.
Treatment and therapy
Rapidly progressive glomerulonephritis is an emergency and must be treated promptly. Usually, the cause of the disease is not yet known when therapy is initiated. Patients with type 1 RPGN benefit from plasmapheresis. This involves plasma exchange. Here, the harmful antibodies are removed from the blood plasma. This can often stop the inflammatory process. If patients develop anuria, temporary dialysis may be required. While simple glomerulonephritis responds well to glucocorticoid therapy, monotherapy with glucocorticoids is not promising in RPGN. In most cases, additional administration of cytostatics and immunosuppressants is necessary.
Prevention
Because the exact mechanisms of development of rapidly progressive glomerulonephritis are still unknown, the disease cannot be prevented. Early diagnosis is very important because of its rapid and severe progression.
Follow-up
The course of rapidly progressive glomerulonephritis can be improved in follow-up as well as in prevention by dietary changes. Patients can reduce the risk of renal failure with a balanced diet and adequate fluids. Close consultation with the physician is helpful in this regard. The regular examination appointments serve to quickly detect any complications. This is an important basis for early reactions. Following the actual therapy, a health-conscious approach is advisable. This includes taking the recommended medication. Other measures to regulate blood pressure also support follow-up care. During the recovery phase, physiotherapy is helpful. This stimulates the metabolism and thus alleviates the symptoms at the same time. If a kidney transplant has taken place due to an already advanced disease, patients should take it easy afterwards. In this case, the bed rest recommended by the doctor must be observed. Good personal hygiene is also part of comprehensive aftercare. When considering whether medications from natural medicine will help against the pain, the doctor should definitely be consulted. Medical specialists are familiar with the mode of action of alternative painkillers. That is why it is also very important to always follow the doctor’s advice.
This is what you can do yourself
Rapidly progressive glomerulonephritis must always be treated by a doctor. Medical therapy can be accompanied by various self-help measures by patients. First of all, a change in diet is important. A healthy and balanced diet may still be able to avert the threat of chronic renal insufficiency. In addition, the patient must consult the doctor regularly to ensure that the disease does not take a serious course or so that the necessary measures can be taken immediately in the event of serious complications. Drinking regularly and taking medications that regulate blood pressure and blood thickness are also important for a speedy recovery. Self-sufferers can also engage in physiotherapy to boost metabolism and thereby relieve pain. In the case of advanced disease, kidney transplantation may be the only option. After a surgical procedure, bed rest and sparing apply. In addition, strict personal hygiene must be observed to prevent the surgical wound from becoming infected. In consultation with the doctor, alternative painkillers from the field of naturopathy can be taken, for example devil’s claw or valerian preparations.
