Fibrillary glomerulonephritis (GN) is a disease that affects the millions of filtering units that constitute the kidney. These filtering units are called glomeruli. Each glomerulus is composed of multiple layers of straining material. Moreover, the body makes a lot of abnormal proteins in this condition. The filters receive these proteins. Therefore, filtration gets disrupted as a result of these proteins getting stuck in the straining layers.
In some instances, these abnormal proteins activate the immune system. This leads to swelling in these delicate structures. The layers of the filters could be damaged by the swelling over time which can lead to the leakage of proteins into the urine. The filtering components become irreparably harmed as the swelling progresses and gets replaced by scar tissue. With insufficient filtering channels, the kidneys lose the capacity to channel and clean the blood. Therefore, end-stage kidney disease is the name given to this problem.
Who Gets Fibrillary GN?
This disease has been found in patients aged 10 to 89. It appears to affect Caucasians at a slightly higher rate. There may be an association of this disease with conditions of immune system dysfunction, such as lupus. This disease can also be associated with conditions causing chronic immune system activation, such as Hepatitis C. Patients with a history of the following conditions are at a greater risk for fibrillary GN-
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Leukemia- Cancer of the blood-forming tissues.
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Multiple Myeloma- Cancer of a type of white blood cell called plasma cells.
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Crohn’s Disease- Type of inflammatory bowel disease affecting the digestive tract lining.
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Lupus- Immune system attacks the body’s own tissues and organs.
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Graves’ Disease- Autoimmune disorder causing overproduction of thyroid hormone.
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Immune Thrombocytopenia (ITP)- Low levels of platelets causing excessive bleeding.
What Are the Symptoms of Fibrillary GN?
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The individual filtering units of the kidney are the first to be affected by fibrillary GN. The symptoms that develop are linked to this first site of damage. The blood components are then excreted in the urine.
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Swelling typically occurs in the legs or feet.
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Due to a significant loss of protein in the urine, affected individuals also experience foamy urine. However, without swelling, patients frequently develop protein in their urine. When their primary care physician performs routine urinalysis, they become aware of their condition for the first time when protein is found in their urine.
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Red blood cells may also pass through the filters into the urine. Most of the time, there are not enough of them to see with the naked eye. A doctor should conduct a microscopic examination of the urine to confirm the presence of blood identified by urinalysis.
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Individual filtering units are lost as kidney damage progresses.
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Numerous patients foster hypertension, regularly requiring different drugs for control.
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Phosphorus balance may be difficult for patients to control.
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Anemia (lack of or dysfunctional red blood cells) is a common problem for patients. Erythropoietin can no longer be secreted by the kidney, resulting in this condition. This hormone stimulates the production of red blood cells in the bone marrow.
Fibrillary GN tends to be a progressive disease. It ultimately leads to loss of kidney function and the need for dialysis or kidney transplant.
Symptoms of advanced kidney failure include-
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Fatigue.
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Confusion.
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Itching.
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Loss of appetite.
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Funny taste in the mouth.
What Are the Related Disorders?
Some of the disorders that are relative to fibrillary GN include the following-
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Immunotactoid glomerulopathy is a similar condition in which the glomerulus is affected by antibodies (immunoglobulins) and microtubules rather than fibrils.
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Fibronectin glomerulopathy is a similar condition in which the glomerulus, rather than the fibrils, are affected by a protein called fibronectin-1.
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Collagenofibrotic glomerulopathy is also a similar disease in which collagen affects the glomerulus rather than the fibrils.
What Types of Tests Detect Fibrillary GN?
Routine laboratory tests can detect early signs of kidney damage. For instance,
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Urinalysis may reveal protein or blood loss in the urine.
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A microscopic examination of the urine can reveal additional kidney involvement.
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A marker of kidney function can rise later in the disease, indicating the loss of functioning filters.
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However, the findings mentioned above do not always identify this disease because there are numerous conditions that can damage the filters.
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A microscopic examination of the kidney tissue is required to make a diagnosis of fibrillary GN. If the tissue is examined, there may be evidence of protein buildup in the filters.
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In the end, electron microscopy is required to identify the kind of proteins that are present. Numerous blood tests are frequently performed as part of the examination to check for other renal diseases or conditions that are linked to this disease.
What Are the Treatment Options for Fibrillary GN?
Currently, there is no proven treatment for this disease. There have been numerous unsuccessful treatments.
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Agents that alter the immune system have been used in the past as treatments. Steroids, Cyclosporine, and Cyclophosphamide are some of these agents.
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Physicians have also tried plasmapheresis in very specific situations. The blood is filtered outside the body in an effort to remove proteins during this procedure.
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After testing for associated conditions like lupus or hepatitis, the treatment must be decided on. A thorough examination of the kidney sample and an assessment of kidney function are required. Before use, the risks associated with suppressing agents' effects on the immune system must be considered.
Conclusion:
Fibrillary glomerulonephritis is an uncommon kidney disorder affecting the glomerulus or the filtering units of the kidneys. It is a progressive disease that may ultimately lead to kidney failure. For the treatment of fibrillary glomerulonephritis with an unknown cause, there is currently no approved treatment. Instead, doctors will probably concentrate on treating the symptoms. The treatment of the underlying condition, which may alleviate symptoms of fibrillary glomerulonephritis, will be the primary focus of clinicians if an underlying condition is present. Even though fibrillary glomerulonephritis can recur after a kidney transplant, patients who progress to end-stage renal disease will likely require dialysis or a kidney transplant.
