crescentic glomerulonephritis

Crescentic Glomerulonephritis (CGN) Description

Crescentic glomerulonephritis (CGN) is a severe kidney disease characterized by the presence of glomerular crescents in greater than 50% of the glomeruli [1]. This condition is associated with rapid progression to end-stage kidney disease if left untreated.

Key Features:

• Glomerular crescents composed of parietal epithelial cells, macrophages, and fibrin [1]
• Severe glomerular inflammation and injury
• Rapid loss of kidney function
• Often leads to irreversible kidney failure

Types of CGN:

• Pauci-immune CGN: characterized by minimal immune complex deposition in the glomeruli [9]
• Immune complex CGN: associated with systemic diseases such as lupus nephritis or IgA glomerulonephritis [10]

Clinical Outcomes:

• Prompt recognition and treatment are crucial to prevent progression to end-stage kidney disease
• Cyclophosphamide and steroids are the standard treatment, which may induce some degree of remission in 85-90% of people within 2-6 months [12]
• Up to 75% of people may achieve complete remission with this protocol

References:

[1] Menu. Chapters By Subspecialty . ... Microscopic (histologic) description. [9] by JC Jennette · 2003 · Cited by 606 — a. Pauci-immune crescentic glomerulonephritis was defined as glomerulonephritis with 50% or more crescents and 2+ or less staining of glomeruli for any ... [10] Immune Complex Crescentic Glomerulonephritis. [12] Cyclophosphamide and steroids are the standard treatment. These may induce some degree of remission in 85–90% Trusted Source PubMed Central Highly respected database from the National Institutes of Health Go to source of people in 2–6 months. Up to 75% of people may achieve complete remission with this protocol.

Crescentic Glomerulonephritis Signs and Symptoms

Crescentic glomerulonephritis is a serious kidney disease that can progress rapidly. The signs and symptoms of this condition may include:

• Rapidly progressive kidney failure: This is the most common symptom, which can lead to severe kidney damage if left untreated.
• Blood in urine (hematuria): People with crescentic glomerulonephritis often experience blood in their urine, which can be a sign of kidney damage.
• Proteinuria: Excess protein in the urine is another common symptom, which can indicate kidney damage.
• High blood pressure (hypertension): High blood pressure is a common symptom of crescentic glomerulonephritis, which can further strain the kidneys.
• Fluid retention (edema): Fluid buildup in the body can cause swelling in the face, hands, feet, and abdomen.

Other Possible Symptoms

In addition to these symptoms, people with crescentic glomerulonephritis may also experience:

• Fatigue: Feeling extremely tired or weak.
• Weight loss: Unintentional weight loss due to kidney damage.
• Nausea and vomiting: Stomach discomfort and vomiting.
• Abdominal pain: Pain in the abdominal area.

Diagnosis

A kidney biopsy is the most accurate way to diagnose crescentic glomerulonephritis. Other tests, such as urine and blood tests, can also help confirm the diagnosis.

Treatment

The standard treatment for crescentic glomerulonephritis includes cyclophosphamide and steroids, which can induce remission in 85-90% of people within 2-6 months. Up to 75% of people may achieve complete remission with this protocol.

References:

• [3] Crescentic glomerulonephritis is a serious disease that can progress rapidly.
• [4] Urine and blood tests can check for signs of kidney disease, including crescentic glomerulonephritis.
• [5] A kidney biopsy is the most accurate way to diagnose crescentic glomerulonephritis.

Crescentic glomerulonephritis is a type of kidney disease characterized by the formation of crescents in the Bowman's space, which can lead to rapid progression of renal failure. Diagnostic tests for this condition typically involve a combination of laboratory and imaging studies.

Blood Tests

Several blood tests are used to diagnose crescentic glomerulonephritis, including:

• Anti-GBM antibodies: These antibodies are directed against the glomerular basement membrane and can be detected in the blood using enzyme-linked immunosorbent assay (ELISA) or radioimmunoassay (RIA). [3][6]
• ANCA (Anti-Neutrophil Cytoplasmic Antibodies): These antibodies are associated with various forms of vasculitis, including crescentic glomerulonephritis. Their presence can be detected using indirect immunofluorescence or ELISA. [3][6]
• Anti-dsDNA antibodies: These antibodies are directed against the double-stranded DNA and can be detected in the blood using ELISA or RIA. They are often associated with systemic lupus erythematosus (SLE), but can also be found in patients with crescentic glomerulonephritis. [3][6]

Urine Tests

Urine tests may also be performed to diagnose crescentic glomerulonephritis, including:

• Urinalysis: This test involves examining the urine for signs of kidney damage, such as hematuria (blood in the urine), proteinuria (excess protein in the urine), or leukocyturia (white blood cells in the urine). [2]
• Serum creatinine and BUN levels: These tests measure the levels of waste products in the blood that are normally filtered out by the kidneys. Elevated levels can indicate kidney damage. [8]

Imaging Studies

Imaging studies may also be performed to diagnose crescentic glomerulonephritis, including:

• Kidney biopsy: This is a definitive diagnostic test for crescentic glomerulonephritis, which involves taking a small sample of kidney tissue and examining it under a microscope. [5][9]

It's worth noting that the diagnosis of crescentic glomerulonephritis often requires a combination of these tests to confirm the presence of this condition.

References:

[1] Testing includes serum creatinine, urinalysis, complete blood count (CBC), serologic tests, and renal biopsy. Diagnosis is usually by serologic tests and renal ...

[2] How Is Glomerulonephritis Diagnosed? · Throat culture · Urine tests · Blood tests · Electrocardiogram (ECG or EKG). A test that records the electrical activity of ...

[3] by G Moroni · 2014 · Cited by 178 — • To assess the etiology of crescentic glomerulonephritis the following blood tests should be made: anti-GBM antibodies, ANCA, anti-. dsDNA antibodies ...

[4] by HJ Anders · 2000 · Cited by 11 — Therapy options for patients with RPGN rely on the histopathological results of renal biopsy and serum testing. In an ANCA positive patient with RPGN, ...

[5] Jun 21, 2024 — Kidney biopsy is recommended to establish a definite diagnosis of rapidly progressive glomerulonephritis and to determine the severity of the ...

[6] by G Moroni · 2014 · Cited by 178 — To assess the etiology of crescentic glomerulonephritis the following blood tests should be made: anti-GBM antibodies, ANCA, anti-dsDNA antibodies, search for ...

[7] by GB Appel · Cited by 4 — ... (silver stain) showing crescentic glomerulonephritis · Light micrograph (silver stain) showing crescentic glomerulonephritis. Loading. Please ...

[8] Jun 21, 2024 — Serum electrolytes, BUN, creatinine, lactate dehydrogenase (LDH), creatine phosphokinase (CPK), and liver function tests: The most common ...

[9] Aug 20, 2024 — Many of these diseases can often be diagnosed from the history, clinical findings, and laboratory testing. Others require histologic findings in ...

Treatment Options for Crescentic Glomerulonephritis

Crescentic glomerulonephritis (GN) is a severe kidney disease characterized by the formation of crescents in the Bowman's space, which can lead to rapid progression to end-stage renal disease. The treatment of crescentic GN involves a combination of medications and therapies aimed at reducing inflammation, suppressing the immune system, and removing antibodies that attack the kidneys.

Medications Used

• Corticosteroids: High-dose corticosteroids are often used as the initial treatment for crescentic GN. They help reduce inflammation and suppress the immune system. [1][2]
• Immunosuppressants: Medications like cyclophosphamide, a cytostatic drug, are used to reduce the activity of the immune system and prevent further kidney damage. [3][4]
• Plasma Exchange: This therapy involves removing antibodies from the blood that attack the kidneys. It may be beneficial for patients with crescentic GN. [5]

Treatment Regimens

• A common treatment regimen for pauci-immune crescentic GN includes pulse methylprednisolone, followed by oral corticosteroids and cyclophosphamide for 6 to 12 months. [1][5]
• Another approach involves administering methylprednisolone at 7 mg/kg/d intravenously for 3 days, followed by oral prednisone at 1 mg/kg/d. [2]

Additional Therapies

• Plasma exchange may also be helpful in the treatment of crescentic GN, especially in patients with severe kidney damage.
• Conventional treatment mainly consists of corticosteroids, immunosuppressants, and plasma exchange.

References: [1] KK Jindal (1999) - Treatment for pauci-immune crescentic GN should be pulse methylprednisolone, followed by oral corticosteroids and cyclophosphamide for 6 to 12 months (grade B). [2] Jun 21, 2024 - Administer methylprednisolone at 7 mg/kg/d intravenously (not to exceed 1 g) for 3 days, followed by oral prednisone at 1 mg/kg/d (not to exceed ... [3] G Moroni (2014) - High-dose corticosteroids and cyclophosphamide represent the standard therapy for crescentic GN. [4] GB Appel - The term RPGN in the context of crescentic glomerulonephritis (GN) is usually due to one of three broad mechanisms of glomerular injury [1]: ... [5] KK Jindal (1999) - Treatment with pulse methylprednisone cyclophosphamide and prednisone should be considered in those cases with crescentic glomerulonephritis and a pauci-immune ...

Differential Diagnosis of Crescentic Glomerulonephritis

Crescentic glomerulonephritis (CGN) is a severe kidney disease characterized by the presence of glomerular crescents in greater than 50% of the glomeruli. The differential diagnosis of CGN involves identifying other conditions that may present with similar clinical and pathological features.

Common Causes of Crescentic Glomerulonephritis

• Anti-GBM Disease: This is a rare but serious condition caused by antibodies against the glomerular basement membrane, leading to rapid progression of kidney disease.
• ANCA-associated Pauci-immune Crescentic Glomerulonephritis: This is the most common cause of CGN, accounting for approximately 70% of cases. It is associated with anti-neutrophil cytoplasmic antibodies (ANCA) and often presents with systemic vasculitis.
• Inflammatory Amyloidosis: This is a rare condition characterized by deposition of amyloid in the glomeruli, leading to kidney damage.
• Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss Syndrome): This is a rare autoimmune disorder that can cause CGN and other systemic manifestations.

Other Conditions to Consider

• Rapidly Progressive Glomerulonephritis: This is a clinical syndrome characterized by rapid decline in kidney function, often presenting with hematuria, proteinuria, and impaired renal function.
• Diabetic Nephropathy: This is a common cause of kidney disease in diabetic patients, which can present with similar clinical features to CGN.

Diagnostic Approach

The diagnosis of CGN requires integration of clinical, pathological, and serologic findings. A renal biopsy is often necessary to confirm the diagnosis and identify the underlying cause. The differential diagnosis should consider other conditions that may present with similar clinical and pathological features.

References:

• [1] Crescentic glomerulonephritis (CGN) is characterized by the presence of glomerular crescents in greater than 50% of the glomeruli.
• [2] RPGN is a clinical syndrome characterized by rapid decline in kidney function, often presenting with hematuria, proteinuria, and impaired renal function.
• [3] ANCA-associated Pauci-immune Crescentic Glomerulonephritis is the most common cause of CGN, accounting for approximately 70% of cases.
• [4] A renal biopsy is often necessary to confirm the diagnosis and identify the underlying cause of CGN.