Rapidly progressive glomerulonephritis is a special type of glomerulonephritis. Typical of the rapidly progressive glomerulonephritis is the rapid increase in so-called retention values in patients suffering from glomerulonephritis. The phenomenon represents a medical emergency situation. If not treated, the life of the sick person is at risk due to a failure of the kidney function.
What is rapidly progressive glomerulonephritis?
Rapidly progressive glomerulonephritis is referred to by many medical professionals as RPGN. The disease manifests itself in flares of extracapillary glomerulonephritis. In many cases, the rapidly progressive glomerulonephritis develops into kidney failure due to the insufficiency of the performance of the organ. See AbbreviationFinder for abbreviations related to Rapidly Progressive Glomerulonephritis.
For this reason, rapid and individually tailored treatment of rapidly progressive glomerulonephritis is essential, otherwise patients will experience complete renal failure within a few weeks or months. Rapidly progressive glomerulonephritis is sometimes referred to as crescentic glomerulonephritis.
Currently, rapidly progressive glomerulonephritis is relatively rare, with a prevalence of less than 1:1,000,000. The exact cause of rapidly progressive glomerulonephritis cannot be determined in every case. There are basically three different forms of rapidly progressive glomerulonephritis, which are primarily distinguished from one another by histological analyses.
Causes
Rapidly progressive glomerulonephritis often presents as an idiopathic disease with no specific cause identified. In other cases, the rapidly progressive glomerulonephritis results from systemic diseases, such as granulomatosis with polyangiitis or a so-called microscopic polyangiitis.
In the first type of rapidly progressive glomerulonephritis, the causes are mainly found in certain antibodies. These are directed against the glomerular basal membrane. The phenomenon occurs, among other things, in the so-called Goodpasture syndrome. The presence of the typical antibodies on histological examination indicates this type of rapidly progressive glomerulonephritis.
The second form of the disease is triggered by certain immune complexes accumulating and depositing. Such a development is possible, for example, in lupus erythematosus. Rapidly progressive type II glomerulonephritis also develops in other pathogenes associated with immune complexes.
The third type of disease, rapidly progressive glomerulonephritis, is characterized by the fact that neither antibodies against basal membranes nor immune complexes are identified on electron microscopic examinations. Some people have vasculitis associated with Wegener’s disease. Microscopic polyangiitis is also possible. This form of the disease is also sometimes called Pauci immune glomerulonephritis.
Symptoms, Ailments & Signs
Rapidly progressive glomerulonephritis begins suddenly and progresses rapidly. The sick people suffer, for example, from high blood pressure and proteinuria. In some cases, the nephrotic syndrome appears.
Kidney weakness often develops quickly, which, if left untreated, often leads to kidney failure. Therefore, patients with rapidly progressive glomerulonephritis usually require dialysis shortly after the onset of the disease. However, the kidneys do not appear enlarged on imaging studies.
When rapidly progressive glomerulonephritis occurs in association with Goodpasture syndrome, people may experience pulmonary hemorrhage. Thromboses on the glomeruli become visible in microscopic examinations of histological tissue samples. In addition, characteristic crescents can be seen in this area.
Diagnosis & course of disease
The diagnosis of rapidly progressive glomerulonephritis is made by a specialist doctor. Serological analyzes play an important role in this. Crucial markers are, for example, antibodies against the basal membranes or so-called ANCAs. In addition, a biopsy of the kidney is usually performed.
In the majority of cases, the course of the rapidly progressive glomerulonephritis resembles the acute nephritic syndrome and causes corresponding typical symptoms that facilitate diagnosis. The histological examination shows, among other things, a crescent of the glomeruli. The neighboring areas are often inflamed.
The different types of rapidly progressive glomerulonephritis can be identified using special staining techniques. Such methods are called immunofluorescence and, together with the other symptoms, ensure the diagnosis of rapidly progressive glomerulonephritis.
Complications
First and foremost, those affected by this disease suffer from very high blood pressure. This can often lead to bleeding and also to a heart attack. The disease also leads to kidney weakness, which in the worst case can lead to kidney failure.
The patients are dependent on a transplant or on dialysis in order not to die. Bleeding occurs in the lungs, which can cause difficulty breathing. The patient’s quality of life is significantly reduced and restricted by the disease. People may also lose consciousness due to difficulty breathing and potentially injure themselves if they fall.
The patients also appear very tired and exhausted and no longer actively participate in everyday life. Psychological problems or depression can also occur as a result of this disease. The treatment itself is carried out with the help of drugs. This significantly reduces the discomfort.
However, most of those affected depend on a donor kidney to stay alive. Complications can also arise during translation. As a rule, the life expectancy of the patient with this disease is significantly reduced.
When should you go to the doctor?
This disease must always be treated by a doctor. Early diagnosis and treatment of glomerulonephritis have a positive effect on the further course of the disease. This can also prevent further complications. As a rule, glomerulonephritis does not heal itself, and the disease cannot be treated with self-help either.
A doctor should be consulted if the person concerned suffers from severe high blood pressure. This blood pressure occurs without a recognizable reason. Bleeding can even occur in the lungs, so the saliva can taste bloody. If these symptoms occur, a visit to a doctor is definitely necessary. Spots can also form on the skin, which could also indicate the disease. In most cases, glomerulonephritis can be diagnosed by a general practitioner. Furthermore, in an emergency, an ambulance should be called immediately or the hospital should be visited.
Treatment & Therapy
The treatment of rapidly progressive glomerulonephritis is primarily drug-based. Patients receive high doses of immunosuppressive drugs. At the same time, people take steroids and the substance cyclophosphamide. If Goodpasture’s syndrome is present, plasma separation is also used.
As soon as the patients suffer from kidney failure, regular dialysis is necessary. If an underlying disease of the rapidly progressive glomerulonephritis is known, its treatment is the priority. In the majority of cases, dialysis is urgently needed because the patient’s life is in danger.
The prognosis of rapidly progressive glomerulonephritis benefits from an early start of technical treatment measures. If people do not respond adequately to the measures, a kidney transplant can be considered. If the therapeutic intervention is too late, the affected patient may die as a result of kidney failure.
Prevention
According to the current state of knowledge, prevention of the rapidly progressive glomerulonephritis is hardly possible. The causes of the development of rapidly progressive glomerulonephritis are not exactly known, so that effective countermeasures before the onset of the disease have not been sufficiently researched.
Aftercare
Follow-up treatment for rapidly progressive glomerulonephritis consists primarily of preventing disease that may develop as a result of rapidly progressive glomerulonephritis or dialysis. To prevent potassium deficiency or other nutrient deficiencies, the blood must be checked regularly. In addition, dietary supplements should be taken after dialysis that contain all the nutrients that can be lost through dialysis.
If a deficiency is detected in the blood, additional nutrient-rich infusions should be administered. In addition, blood pressure should be checked regularly. If blood pressure is found to be too high, blood pressure-lowering medication and, if necessary, beta-blockers must be taken. If the kidneys are still working, flushing medication (water tablets) should be taken as a precaution, as pulmonary edema and pulmonary bleeding can occur as a result of rapidly progressive glomerulonephritis.
In this case, the kidney values should also be checked regularly in order to be able to recognize and treat complete kidney failure in good time. In addition, the lungs should be examined regularly using imaging methods (CT, MRT, ultrasound) in order to detect accumulations of water at an early stage. If shortness of breath occurs, a doctor must be consulted immediately, since a pulmonary edema that has developed could be the cause.
Alcohol consumption and smoking must be stopped after rapidly progressive glomerulonephritis because the toxins in alcohol and tobacco can overload the body’s weakened detoxification function, resulting in death.
You can do that yourself
Rapidly progressive glomerulonephritis is a medical emergency and needs to be treated quickly. Otherwise there is a risk of chronic renal failure. Self-help is not possible in the acute stage of the disease. All necessary medical measures must be taken to avoid chronic nephritic syndrome. However, this is not always possible. Much depends on the timing of the start of treatment.
However, if chronic kidney failure develops despite therapy, there are a number of self-help options that can be used to stop or slow down the disease process. However, it should be borne in mind that renal insufficiency is a term that summarizes many different kidney diseases and disease stages. Therefore, it helps the patient to exchange ideas with other sufferers within self-help groups. Addresses of self-help groups can be found on the Internet, among other things. The exchange of information about self-help options, preventive measures and experiences helps many patients to cope better with everyday life and thus improve their quality of life. This applies to both physical and mental health. Already the certainty
The patient’s personal responsibility includes, among other things, familiarizing himself with information about the clinical picture, recognizing risk factors, visiting the nephrologist regularly, changing his diet, constantly checking his blood pressure and getting plenty of fresh air.
