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Membranoproliferative Glomerulonephritis - What Is the Significance?

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Membranoproliferative glomerulonephritis is a kidney disease that involves inflammation and changes in renal cells in the kidneys. Read on to learn more.

Medically reviewed byDr. Yash Kathuria

Published At March 21, 2023
Reviewed AtNovember 1, 2023

Introduction:

Glomerulonephritis is inflammation of the glomeruli. The kidney glomeruli help filter waste and fluids from the blood to form urine. Damage to this membrane impairs the ability of the kidneys to produce urine normally. Blood and protein may leak into the urine. When enough protein is present in urine, fluid leaks from blood vessels into body tissues, causing swelling (edema). Nitrogenous waste products can also build up in the blood (azotemia).

What Is Membranoproliferative Glomerulonephritis?

Many diseases can affect kidney function by attacking and damaging the glomeruli (the small filtering units of the kidneys that clean the blood), called glomerular diseases. They include many diseases with different causes. One such disease is membranous proliferative glomerulonephritis (MPGN). MPGN occurs when the body's defense system is not working properly. The body's defense system is called the immune system. MPGN is of two different types, based on how the immune system damages the kidney and which part of the glomerulus is damaged. The two forms are MPGN I and MPGN II. Most people with this disease are type I. MPGN II is very rare. It also tends to deteriorate faster than MPGN I.

What Are the Causes of Membranoproliferative Glomerulonephritis?

With MPGN, the immune system begins attacking healthy cells in the kidneys that damage the glomeruli. The immune system produces proteins called antibodies to attack substances that the body considers harmful. Contaminants are called antigens. Antigens combine with antibodies to form immune complexes. These immune complexes lodge in the kidneys and cause damage.

What Are the Risk Factors of Membranoproliferative Glomerulonephritis?

People with the following conditions are at an increased risk of developing MPGN:

1. Autoimmune

  • Systemic lupus erythematosus (SLE).

  • Sjögren syndrome.

  • Rheumatoid arthritis.

  • Inherited complement deficiencies (in particular, C2 deficiency).

  • Scleroderma (a disease that involves the tightening and hardening of the skin).

  • Celiac disease (immune reaction of the body from eating gluten).

2. Chronic Infections

  • Viral – Hepatitis B, cryoglobulinemia type II, hepatitis C.

  • Protozoal – Malaria, schistosomiasis.

  • Bacterial – Endocarditis, infected ventriculoatrial (or jugular) shunt, leprosy, multiple visceral abscesses.

3. Malignant Neoplasms

  • Lymphoma.

  • Carcinoma and leukemia are associated with a membranoproliferative pattern of renal injury.

What Are the Symptoms of Membranoproliferative Glomerulonephritis?

People suffering from membranoproliferative glomerulonephritis notice the following symptoms.

1. Hematuria - Glomerular disease causes the glomeruli to leak blood into the urine. The urine may appear pink or light brown with blood in it.

2. Urinary Protein - Glomerular disorders can cause the glomeruli to leak protein into the urine. Urine may be foamy due to protein.

3. Edema - Glomerular disease can cause fluid accumulation. Excess fluid can cause swelling in body parts such as the hands, ankles, or around the eyes.

4. Nephrotic Syndrome- It is a syndrome with a set of symptoms that together affect the kidneys and these include:

  • Swelling (edema) in body parts, such as the feet, ankles, or around the eyes.

  • Presence of a high amount of protein in the urine (proteinuria).

  • Loss of protein in the blood.

  • High fatty lipids (high cholesterol) in the blood.

  • High blood pressure.

When to Get Help for Membranoproliferative Glomerulonephritis?

Call the emergency services as soon as possible if any of the following symptoms arise:

  • If a person is having symptoms of membranoproliferative glomerulonephritis.

  • If the symptoms get worse or do not go away.

  • New symptoms, such as decreased urine output arise.

How Is Membranoproliferative Glomerulonephritis Diagnosed?

The following tests are done to detect membranoproliferative glomerulonephritis:

  • Urinalysis - Urinalysis helps detect protein and blood in the urine.

  • Blood Test - Blood tests help measure levels of protein, cholesterol, and waste products in the blood.

  • Glomerular Filtration Rate (GFR) - Blood tests are done to determine how well are the kidneys filtering waste products from the body.

  • Renal Biopsy - For this test, part of the kidney is cut and removed with a special needle and viewed under a microscope. It is very important that the person performing the biopsy is an expert in glomerular disease because MPGN is very rare and knowing the class of the disease helps doctors determine the best treatment. These types of experts may be found in large research centers.

  • Genetic Testing - Tests may be done to determine if MPGN is caused by genes inherited from the family.

What Is the Treatment of Membranoproliferative Glomerulonephritis?

The general treatment plan remains similar for adults and children. The goal of treatment is to give certain types of drugs to prevent the immune system from damaging the kidneys. There is no cure for this disease. Treatment focuses on controlling symptoms and slowing disease progression. Before creating a treatment plan, doctors try to find the cause of MPGN. Unless MPGN is caused by another illness, such as hepatitis C, the treatment regimen will be different. Treatment with many drugs can help slow the progression of the disease and control symptoms such as high blood pressure, proteinuria, and edema. The following medications may be given to slow the process of kidney damage:

  • Corticosteroids and Immunosuppressants - These drugs are used to boost the immune system, or the body's defense system, and calm it down. Preventing attack on the glomerulus.

  • ACE Inhibitors and ARBs - These are blood pressure medications that reduce protein loss and control blood pressure.

  • Diet Changes - Some dietary changes may be required. Reducing salt (sodium) and protein intake in the diet helps reduce the burden on the kidneys.

What Are the Complications of Membranoproliferative Glomerulonephritis?

Common complications of MPGN include:

  • End-stage renal disease (ESRD).

  • Edema.

  • Periorbital dependent edema.

  • Infection with encapsulated bacteria like (Haemophilus species, Streptococcal species, Klebsiella species).

  • Thromboembolism.

Several predisposing factors may increase susceptibility to thromboembolism. These factors include:

  • Decrease in anticoagulant factors such as protein C and S and antithrombin III.

  • Increased platelet aggregation.

  • Increase procoagulant proteins.

  • Hyperlipidemia.

  • Fibrinolytic disorders.

  • Malnutrition.

  • Iron deficiency anemia.

  • Hypocalcemia due to hyperparathyroidism secondary to vitamin D deficiency.

How to Prevent Membranoproliferative Glomerulonephritis?

Some types of glomerulonephritis cannot be prevented. However, here are some steps that may be beneficial:

  • Seek immediate treatment for Streptococcal infections with sore throat and impetigo.

  • To prevent infections that can lead to certain types of glomerulonephritis, such as HIV (human immunodeficiency virus) and hepatitis, follow safe sex guidelines and avoid injecting drugs.

  • Medication for hypertension control high blood pressure and reduces the chance of kidney damage due to high blood pressure.

Conclusion:

Membranoproliferative glomerulonephritis (MPGN) is a type of glomerulonephritis caused by an abnormal immune response in the body. Antibody deposits accumulate in a part of the kidney called the glomerular basement membrane. This membrane helps filter waste and excess water from the blood. This condition mostly affects children. In many cases, the condition slowly worsens and eventually leads to chronic renal failure. Half of the people with this condition develop chronic renal failure within ten years. This more likely occurs in people with high protein levels in their urine. A regular medical checkup is important for a timely diagnosis of this condition.

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Frequently Asked Questions

Membranoproliferative glomerulonephritis is classified as type 1 and type 2. Among these, type 1 is the most common. Type 1 membranoproliferative glomerulonephritis is also known as immune complex-mediated MPGN. It is characterized by the presence of immune complexes (antibodies bound to antigens) in the glomeruli of the kidneys. The immune complexes activate the complement system, leading to inflammation and damage to the glomerular membrane.

Type I MPGN is often associated with various underlying conditions, such as chronic infections (e.g., hepatitis B and C), autoimmune diseases (e.g., systemic lupus erythematosus), and certain malignancies.

The key differences between membranoproliferative glomerulonephritis and membranous glomerulonephritis lie in the histopathological findings (specifically, the patterns of thickening and proliferation) and the underlying mechanisms of disease (involving immune complexes and complement activation). Both conditions can result in kidney dysfunction, proteinuria (protein in the urine), and, in severe cases, lead to chronic kidney disease or kidney failure.

Membranoproliferative glomerulonephritis (MPGN) is generally considered a chronic and progressive kidney disease. It is not typically curable because the underlying cause or mechanism causing the disease cannot be completely reversed. However, the progression of MPGN can sometimes be slowed down or managed effectively with appropriate treatment, leading to improved quality of life and preservation of kidney function.

Membranoproliferative Glomerulonephritis (MPGN) was classified into three types, often referred to as MPGN type I, MPGN type II, and MPGN type III.


- MPGN Type I: This type is characterized by the presence of immune complexes that form in the glomeruli of the kidneys.


- MPGN Type II (Also Known as Dense Deposit Disease, or DDD): This type is distinguished by the abnormal accumulation of dense deposits within the glomerular basement membrane.


- MPGN Type III: This type is very similar to MPGN Type I but is distinguished by the absence of immune complex deposits in the glomeruli.

Patients with MPGN may present with various symptoms and signs of kidney dysfunction, such as hematuria (blood in the urine), proteinuria (protein in the urine), hypertension (high blood pressure), and edema (swelling), particularly in the legs and ankles. As the disease progresses, it can lead to chronic kidney disease (CKD) and, in severe cases, end-stage renal disease (ESRD), requiring dialysis or kidney transplantation.

MPGN (Membranoproliferative Glomerulonephritis) can have nephritic and nephrotic syndromes features, depending on the specific subtype and stage of the disease. In MPGN, the nephritic features are typically more prominent early in the disease course, with hematuria and proteinuria being common presentations.

Yes, membranoproliferative glomerulonephritis (MPGN) can have a genetic component in some cases, but it is not purely genetic. MPGN is a type of kidney disease that affects the glomeruli, which are the filtering units of the kidneys.

The duration of Membranoproliferative Glomerulonephritis (MPGN) can vary depending on various factors, including the underlying cause and the individual's response to treatment. In some cases, MPGN may have a chronic course, meaning it persists for an extended period, possibly leading to progressive kidney damage. In other cases, with appropriate treatment, it might be possible to slow down the progression of the disease and manage the symptoms effectively.

The morphology of membranoproliferative glomerulonephritis (MPGN) refers to the characteristic changes in the kidney tissue that can be observed under a microscope.


- Type 1 MPGN - Under the microscope, there are thickened glomerular capillary walls, often described as "tram-track" appearance, due to double contours formed by the immune complex deposits.


- Type 2 MPGN - The deposits are electron-dense and appear as ribbon-like or sausage-shaped structures when observed under an electron microscope.


- Type 3 MPGN - The deposits are similar to those seen in type I MPGN but are composed of immunoglobulin complexes with a different appearance under electron microscopy.

In membranoproliferative glomerulonephritis (MPGN), urinalysis is an essential diagnostic tool used to evaluate kidney function and identify urine abnormalities that may indicate this specific kidney disease. During a urinalysis for MPGN, the following parameters are typically assessed:


- Proteinuria.


- Hematuria.


- Glomerular Filtration Rate (GFR).


- Casts.

Membranoproliferative glomerulonephritis (MPGN) can occur at any age, but it is more commonly diagnosed in children and young adults, typically between the ages of 8 and 30 years. However, it can also affect older adults.

The diagnosis of membranoproliferative glomerulonephritis (MPGN) typically involves a combination of clinical evaluation, laboratory tests, and kidney biopsy. There is no single blood test that can definitively confirm MPGN on its own, but certain blood tests can help support the diagnosis and provide important information about the condition.

Some of the commonly used medications for treating MPGN include:


- Corticosteroid.


- Rituximab.


- Angiotensin-converting enzyme inhibitors.


- Immunosuppressants.


- Diuretics.

Membranoproliferative glomerulonephritis (MPGN) is also known by several other names, including:


- Mesangiocapillary glomerulonephritis.


- Mesangiocapillary glomerulonephritis (MCGN).


- Membranoproliferative glomerulonephritis type I and type II (MPGN type I and II).


- Dense deposit disease (DDD) - this term is specifically used for MPGN type II.

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