IgA nephritis is the most common disease of the renal corpuscles. It is counted among the idiopathic glomerulonephritis.
What is IgA nephritis?
Since IgA nephritis rarely causes symptoms, it is usually diagnosed during investigations into other diseases. The laboratory values of the urine findings play an important role.
According to abbreviationfinder, IgA nephritis is a kidney disease that is associated with hematuria and gradually leads to impairment of kidney function. Deposits of immunoglobulin A (IgA) on the kidney corpuscles (glomeruli) are responsible for this. IgA nephritis is also known as Berger’s disease or IgA nephropathy.
It is one of the most common forms of glomerulonephritis. In Europe, kidney disease accounts for around 30 percent of all glomerulonephritis. In Asian countries such as Korea and Japan, the disease percentage is even around 50 percent. IgA nephritis is two to three times more common in men than in women.
The average age of the disease is about 30 years. In principle, however, it can occur at any age. Most diseases are sporadic. Only five percent of all cases of illness tend to run in families.
Causes
The exact causes of IgA nephritis are still in the dark. Doctors suspect hereditary dispositions that are related to the geographic location of the disease. Significantly elevated levels of immunoglobulin IgA1 and immune complexes involving IgA1 are present in the blood serum of most affected individuals.
Therefore, it is believed that IgA is formed in the outer lymphatic mucosal tissues and undergoes a structural change. This can be a consequence of local infections. The IgA and immune complexes found in patients are often structurally altered. The more severe the disease, the more pronounced the changes.
This is usually an altered glycosylation that is less pronounced than with normal IgA. The reduction in binding makes it easier for the defective IgA and IgA immune complexes to attach to the mesangial cells of the renal glomeruli. Due to the increased defective formation of IgA and the IgA immune complexes, these can be deposited more easily in the kidney corpuscles, which ultimately triggers IgA nephritis.
In the process, faulty IgA is bound to the mesangial cells. The deposition of the IgA antibodies in the renal corpuscles results in inflammation, which impairs the filtering function. This means that the filter cells can no longer hold back red blood cells and blood proteins that are excreted in the urine. On the other hand, toxic metabolic products remain in the organism, which leads to health impairments.
Symptoms, Ailments & Signs
IgA nephritis, which usually remains inconspicuous at first, becomes noticeable through painless gross hematuria. Gross hematuria is the visible appearance of blood in the urine. In the run-up, there are unspecific gastrointestinal infections, respiratory infections or pneumonia.
In some cases, however, IgA nephropathy also shows constant microscopic hematuria. The blood in the urine cannot be seen with the naked eye, so a chemical laboratory test must be carried out. There is also the possibility of increased excretion of protein in the urine, which is referred to as proteinuria. On the other hand, a nephrotic syndrome is only rarely seen.
Some patients also develop high blood pressure. Deformed red blood cells (erythrocytes) and erythrocyte casts can be found in the urine. Furthermore, the level of IgA in the patient’s body is increased. In severe cases, there is also a higher creatinine level.
Diagnosis & course of disease
Since IgA nephritis rarely causes symptoms, it is usually diagnosed during investigations into other diseases. The laboratory values of the urine findings play an important role. A urinalysis reveals hematuria, with deformed red blood cells that come from the kidneys, and a small amount of proteinuria.
Sometimes the IgA level in the body also increases, but this is not sufficient proof of a disease. A kidney biopsy is used to confirm the diagnosis. Mesangial changes can be detected in the renal corpuscle. Furthermore, an immunohistochemical detection of IgA deposits is possible. Dead cells indicate severe impairment of the renal corpuscle.
It is also important to make a differential diagnosis from other glomerular diseases in which blood is present in the urine. These primarily include postinfectious glomerulonephritis, thin basement membrane syndrome, and Alport syndrome. If hematuria is the only symptom of IgA nephritis, the course of the kidney disease is usually benign.
In such cases, no special treatment is required. Likewise, a spontaneous improvement in proteinuria is considered an indication of a positive course. However, if IgA nephritis persists over time, there is a risk of hypertension (high blood pressure) and kidney failure.
Complications
In most cases, IgA nephritis is not diagnosed until late because it is usually painless and shows no other visible symptoms. However, there is blood in the urine, which the patient cannot see with the naked eye. Various infections can also occur, such as stomach infections or inflammation of the airways and lungs.
This leads to breathing difficulties, from which the patient can even die in the worst case. Not infrequently, those affected also suffer from high blood pressure and can thus suffer a heart attack. Patients are often also restricted in their everyday lives because they cannot carry out strenuous physical activities.
Complications can occur if IgA nephritis is not treated, resulting in kidney failure, for example. In this case, the person concerned is then dependent on lifelong dialysis or a donor kidney. Treatment of IgA nephritis is carried out without complications and with the help of drugs. The complaints can thus be limited. If inflammation occurs, it is treated with antibiotics. Life expectancy is usually not reduced by IgA nephritis.
When should you go to the doctor?
A doctor’s visit is necessary for a symptom such as blood in the urine. If it occurs repeatedly and without physical exertion, this is a warning from the body. Since the blood is in many cases difficult to see with the naked eye due to the small amount, the person concerned should be more vigilant when going to the toilet after the first finding. In the case of gastrointestinal problems, diarrhea, abdominal pain, constipation or lack of energy, a doctor is needed to clarify the cause. If the usual level of performance drops, an inner weakness sets in or if the person concerned complains of a general feeling of illness, he should consult a doctor.
A diffuse feeling of illness, breathing disorders or a feeling of pressure in the chest must be examined and treated by a doctor. If you are scared or panicking, you should also see a doctor. If high blood pressure sets in or cardiac arrhythmia sets in, a doctor should be consulted. Dizziness, sleep disorders or emotional abnormalities should be presented to a doctor if they occur again. In many cases, these signs hide diseases that need to be treated. In the event of skin changes, the development of edema or swelling on the body, it is advisable to consult a doctor. If the existing symptoms increase in intensity or extent, a doctor should be consulted as soon as possible.
Treatment & Therapy
There is no specific treatment for IgA nephritis. Therapy depends on the extent and severity of the kidney disease. In the early stages, no treatment is given if the creatinine level is normal. If proteinuria persists or hypertension begins, the patient is given drugs such as angiotensin receptor blockers and ACE inhibitors.
One of the cornerstones of basic therapy is lowering blood pressure. Cortisone preparations such as glucocorticoids can also be administered to reduce protein excretion in the urine. This significantly reduces the risk of renal failure requiring dialysis.
The administration of glucocorticoids lasts around six months and is carried out if the proteinuria cannot be improved by taking the other drugs. If there is a rapid loss of kidney function, a combination of glucocorticoids and cyclophosphamide can also be used. However, the data on this treatment variant is still imprecise. The administration of immunosuppressive drugs is viewed rather critically in recent studies.
Outlook & Forecast
The prognosis of IgA nephritis depends on the severity of the disease. In general, however, a good course can be assumed. If the symptoms are mild, no therapy is necessary. However, an annual examination of kidney function, urine composition and blood pressure should then be carried out.
The decisive factor for therapy is the extent of protein excretion (proteinuria) in the urine. If proteinuria is above 0.5 grams/day, treat with angiotensin receptor blockers or ACE inhibitors. One gram/day must not be exceeded because this worsens the prognosis. In this case, the dose of the medication must be increased until the protein excretion in the urine falls below one gram per day.
A much poorer prognosis is observed in the presence of extensive glomerular sclerosis. In 50 percent of these cases, renal insufficiency develops during the course of the disease. Of these, 10 percent of patients develop such a severe form that a kidney transplant is necessary. Even under treatment, complete failure of the kidneys is possible in a few cases, depending on the strength of the autoimmune reactions of the immune system against the kidneys. After the transplant, IgA nephritis can also develop again in 20 to 50 percent of the patients, but the course is much milder. However, the new disease usually occurs more than 10 years after the transplantation.
Prevention
There are no preventive measures against IgA nephritis. So the exact causes of the kidney disease could not be determined.
Aftercare
Most people affected by IgA nephritis usually have no special options for aftercare. Therefore, an early diagnosis should be made for IgA nephritis so that the symptoms of the disease do not worsen. Early diagnosis always has a very positive effect on the further course of the disease.
Most people affected by IgA nephritis are dependent on taking various medications. A correct dosage and regular intake should be observed. If anything is unclear or if you have any questions, always consult a doctor first to avoid complications. In the case of IgA nephritis, regular check-ups by a doctor are also very important so that the situation of the disease is well monitored.
Self-healing cannot occur. Due to the illness, those affected often depend on the help and support of their own family, friends and acquaintances in their lives. Contact with other people affected by the disease can also be useful, as this can often lead to an exchange of information.
You can do that yourself
In the case of IgA nephritis, there are no options for self-help for those affected. It is also usually not possible to prevent the disease, so that treatment can only be symptomatic and not causal.
Since patients very often suffer from inflammation of the lungs or airways as a result of IgA nephritis, these regions must be treated protected will. Especially in the cold seasons, those affected should dress appropriately to avoid discomfort and complications. The gastrointestinal tract is also often affected by infections, so that in many cases patients are dependent on a light and low-fat diet in order not to irritate the stomach and intestines. High blood pressure is usually treated with medication. However, the patient should refrain from strenuous activities or sports and take it easy on the body. Even Stressful situations be avoided, as they can have a negative effect on the course of the disease.
With IgA nephritis, it is advisable to undergo regular medical examinations. The kidneys in particular should be examined carefully.
