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Crescentic Glomerulonephritis: Symptoms, Diagnosis, and Treatment

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A kidney disease called crescent-shaped glomerulonephritis is characterized by a drastic decrease in renal function.

Written by

Dr. Kinjal Shah

Medically reviewed by

Dr. Yash Kathuria

Published At August 24, 2023
Reviewed AtAugust 24, 2023

Introduction:

Histologically, the presence of significant glomerular crescents (typically larger than 50 percent) defines the condition known as crescentic glomerulonephritis (Cr. GN). Because it is followed by a fast deterioration in renal functioning, it is sometimes referred to clinically as rapidly progressive glomerulonephritis (RPGN). Any glomerular condition can cause it, and it is often seen in between four and ten percent of native kidney biopsies. Since end-stage kidney disease (ESKD) develops in the majority of patients within a few weeks to months, the illness's natural course is comparable to a "medical emergency."

What Is Crescentic Glomerulonephritis?

The kidney condition known as crescentic glomerulonephritis (CGN) is characterized by glomeruli that are inflamed and damaged. The kidneys' small glomeruli are in charge of filtering waste materials and extra fluid from the blood. Due to the buildup of immune cells and immune system proteins within the glomeruli in CGN, the lesions or cellular proliferation on the glomeruli take the form of crescent-shaped lesions or cellular proliferation.

These crescents have the potential to restrict blood flow, which would impede kidney function and cause a rapid reduction in renal function. The glomeruli's microscopic appearance, where a concentration of cells takes the form of crescent-shaped formations, is referred to as a "crescentic" look.

What Are the Causes of Crescentic Glomerulonephritis?

The following are some of the causes of CGN:

  • Pauci-Immune CGN: The most typical kind of CGN, Pauci-immune CGN, is linked to ANCA-associated vasculitis (AAV), an autoimmune disease in which the immune system erroneously assaults the tiny blood vessels. Immune deposits in the glomeruli are not seen in Pauci-immune CGN.

  • Immune Complex-Mediated CGN: Immune complexes, which are collections of antigens and antibodies, develop and settle in the glomeruli to cause this type of CGN. IgA nephropathy, post-infectious glomerulonephritis, and lupus nephritis are conditions that can result in immune complex-mediated CGN.

  • Anti-glomerular Basement Membrane (Anti-GBM) Disease: The term "anti-glomerular basement membrane disease" refers to a rare autoimmune syndrome in which the immune system creates antibodies that target the glomerular basement membrane. Rapidly progressive glomerulonephritis (RPGN), a severe and quickly deteriorating condition, can result from anti-GBM illness.

What Are the Symptoms of Crescentic Glomerulonephritis?

Although CGN symptoms might vary, they frequently include the following:

  • Hematuria (urine with blood).

  • Proteinuria (extra protein in the urine).

  • Edema (swelling) in the ankles, the eyes, or the legs.

  • High blood pressure or hypertension.

  • Reduced urine production.

  • Weakness and weary.

  • Nephrotic syndrome (which includes proteinuria, hypoalbuminemia, edema, and excessive cholesterol levels).

How to Diagnose Crescentic Glomerulonephritis?

Medical History and Physical Exam: The healthcare professional takes down any relevant information, such as any symptoms that have been encountered, past illnesses, and family history. In order to determine general health and spot any indications of kidney illness, such as edema, elevated blood pressure, or fluid retention, they also do a physical examination.

Blood Testing: A number of blood tests are carried out to assess kidney function and find any underlying issues that could be present. These tests might consist of the following:

  • Blood urea nitrogen (BUN) and creatinine levels are tested as part of kidney function tests to determine how well the kidneys are able to filter waste products.

  • Complete blood counts, red and white blood cell counts, as well as platelet levels, which might signify inflammation or anemia, are all revealed by CBC test.

  • Antinuclear antibodies (ANA), anti-neutrophil cytoplasmic antibodies (ANCA), and anti-glomerular basement membrane (anti-GBM) antibodies are a few examples of particular antibodies that can be found during blood testing for autoimmune disorders.

Urine Tests: In order to evaluate kidney function and find any anomalies, urine samples are collected and examined. Key urine tests consist of the following:

  • Urinalysis: The presence of blood, protein, and other anomalies is checked for in the urine.

  • Urine Protein-to-Creatinine Ratio: This test assesses the ratio of urine protein to creatinine levels, indicating the degree of proteinuria.

  • Urine Microscopy: Examining the urine sediment under a microscope reveals the presence of red blood cells, white blood cells, and cellular casts, which can indicate kidney injury.

Imaging Studies: To see the kidneys and spot any structural irregularities or symptoms of chronic renal disease, imaging procedures like ultrasound or CT (computed tomography) scan may be utilized.

Kidney Biopsy: To confirm the diagnosis of CGN and identify the precise underlying cause, a kidney biopsy may be carried out. This process involves taking a small sample of kidney tissue using a needle, which is subsequently inspected under a microscope. Making treatment decisions is aided by the biopsy's ability to detect crescents, cellular growth, and immunological deposits.

Depending on the probable etiology of CGN, the diagnostic procedure frequently entails cooperation between medical practitioners, including nephrologists and other specialists.

What Is the Treatment of Crescentic Glomerulonephritis?

The goal of treating crescentic glomerulonephritis (CGN) is to control complications, decrease inflammation, and cure the underlying cause of the disorder. The particular course of treatment is determined by the degree of CGN, the underlying etiology, and unique patient variables. Here are a few typical treatment methods:

  • Immunosuppressive Medicines: Immunosuppressive drugs are frequently administered to decrease inflammation and suppress the immunological response in cases of CGN linked to autoimmune diseases or immune-mediated processes. These drugs may include immunosuppressants like Cyclophosphamide or Rituximab, as well as corticosteroids like Prednisone. Depending on the underlying reason and response to therapy, the drug selection and length of the course of treatment may change.

  • Plasmapheresis: This process includes filtering the blood to get rid of immunological complexes and dangerous antibodies. It might be used in CGN instances linked to certain antibody-mediated illnesses, including severe immune complex-mediated CGN or anti-GBM sickness.

  • Blood Pressure Management: Controlling high blood pressure (hypertension), a frequent consequence of CGN, is important to stop additional kidney injury. Angiotensin-converting enzyme inhibitors (ACE inhibitors) and angiotensin receptor blockers (ARBs) are routinely used medications that lower proteinuria and regulate blood pressure.

  • Supportive Care: This could entail dietary adjustments, such as lowering salt and protein intake, controlling fluid balance, and making sure to get the right nourishment. Additionally, adequate hydration and consistent electrolyte level monitoring are crucial.

  • Dialysis and Kidney Transplantation: Dialysis or kidney transplantation may be required in extreme situations when CGN causes end-stage kidney disease (ESKD) and a significant reduction in kidney function. While kidney transplantation includes replacing damaged kidneys with healthy kidneys from a donor, dialysis entails using a machine to remove waste materials and extra fluid from the blood.

Conclusion:

The only effective therapy for crescentic GN now available is often immunosuppressive medicines, which have a number of major adverse effects (mostly infections, cancer, and cardiovascular issues). As a result, there is a need for new, safer treatments. It is essential to closely monitor patients and follow up with medical specialists to manage CGN effectively and improve patient outcomes.

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Dr. Yash Kathuria
Dr. Yash Kathuria

Family Physician

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