RAPIDLY PROGRESSIVE GLOMERULONEPHRITIS RPGN Flashcards
What is Rapidly Progressive Glomerulonephritis (RPGN)?
RPGN is a clinical syndrome characterized by the rapid loss of kidney function over days to weeks, associated with glomerular disease, presenting with proteinuria, hematuria, and red cell casts. The pathologic hallmark is the presence of crescents on kidney biopsy, also known as crescentic nephritis.
Crescents are formed by the proliferation of epithelial cells in the Bowman’s capsule in response to injury.
How is RPGN classified?
RPGN is classified into three types based on immune complex deposition:
* Type I: Anti-GBM antibody disease (e.g., Goodpasture disease).
* Type II: Immune complex deposition (e.g., lupus, IgA nephropathy).
* Type III: Pauci-immune (e.g., ANCA-associated vasculitis).
The classification helps guide diagnosis and treatment.
What are the causes of RPGN?
RPGN can be caused by:
* Primary RPGN: Anti-GBM disease, immune complex disease, or pauci-immune disease.
* Secondary RPGN: Systemic diseases such as lupus, vasculitis, infections, or malignancies.
Understanding the underlying cause is essential for effective management.
How is RPGN diagnosed?
Diagnosis involves:
* Serologic tests:
* Anti-GBM antibodies: For Goodpasture disease.
* ANCA: For vasculitis (e.g., granulomatosis with polyangiitis).
* Complement levels: Low in lupus, normal in ANCA-associated vasculitis.
* Kidney biopsy: Essential for definitive diagnosis and prognosis.
A kidney biopsy can reveal crescents and other histopathological changes indicative of RPGN.
What is the treatment for RPGN?
Treatment involves aggressive immunosuppression:
* Corticosteroids: First-line therapy.
* Cyclophosphamide: Used for ANCA-associated vasculitis.
* Plasma exchange: For severe cases (e.g., anti-GBM disease, ANCA vasculitis).
* Early intervention is critical to prevent end-stage kidney disease (ESKD).
The choice of treatment may vary based on the specific type of RPGN.
What is the prognosis of RPGN?
Prognosis varies by underlying cause:
* Anti-GBM disease: Poor prognosis if the patient is dialysis-dependent at presentation.
* ANCA-associated vasculitis: Better response to therapy, even if dialysis-dependent.
Prognosis can be significantly improved with early diagnosis and treatment.
What are the key points to remember about RPGN?
- RPGN is characterized by rapid loss of kidney function, often with crescents on biopsy.
- It is classified into three types: anti-GBM disease, immune complex disease, and pauci-immune disease.
- Diagnosis requires serologic tests (e.g., anti-GBM, ANCA) and a kidney biopsy.
- Early immunosuppressive therapy (e.g., corticosteroids, cyclophosphamide, plasma exchange) is critical.
- Prognosis depends on the underlying cause, with anti-GBM disease having a poor prognosis if dialysis is required early.
These key points summarize the essential aspects of RPGN for quick review.
What is Rapidly Progressive Glomerulonephritis (RPGN)?
RPGN is a clinical syndrome characterized by the rapid loss of kidney function over days to weeks, associated with glomerular disease, presenting with proteinuria, hematuria, and red cell casts. The pathologic hallmark is the presence of crescents on kidney biopsy, also known as crescentic nephritis.
Crescents are formed by the proliferation of epithelial cells in the Bowman’s capsule in response to injury.
How is RPGN classified?
RPGN is classified into three types based on immune complex deposition:
* Type I: Anti-GBM antibody disease (e.g., Goodpasture disease).
* Type II: Immune complex deposition (e.g., lupus, IgA nephropathy).
* Type III: Pauci-immune (e.g., ANCA-associated vasculitis).
The classification helps guide diagnosis and treatment.
What are the causes of RPGN?
RPGN can be caused by:
* Primary RPGN: Anti-GBM disease, immune complex disease, or pauci-immune disease.
* Secondary RPGN: Systemic diseases such as lupus, vasculitis, infections, or malignancies.
Understanding the underlying cause is essential for effective management.
How is RPGN diagnosed?
Diagnosis involves:
* Serologic tests:
* Anti-GBM antibodies: For Goodpasture disease.
* ANCA: For vasculitis (e.g., granulomatosis with polyangiitis).
* Complement levels: Low in lupus, normal in ANCA-associated vasculitis.
* Kidney biopsy: Essential for definitive diagnosis and prognosis.
A kidney biopsy can reveal crescents and other histopathological changes indicative of RPGN.
What is the treatment for RPGN?
Treatment involves aggressive immunosuppression:
* Corticosteroids: First-line therapy.
* Cyclophosphamide: Used for ANCA-associated vasculitis.
* Plasma exchange: For severe cases (e.g., anti-GBM disease, ANCA vasculitis).
* Early intervention is critical to prevent end-stage kidney disease (ESKD).
The choice of treatment may vary based on the specific type of RPGN.
What is the prognosis of RPGN?
Prognosis varies by underlying cause:
* Anti-GBM disease: Poor prognosis if the patient is dialysis-dependent at presentation.
* ANCA-associated vasculitis: Better response to therapy, even if dialysis-dependent.
Prognosis can be significantly improved with early diagnosis and treatment.
What are the key points to remember about RPGN?
- RPGN is characterized by rapid loss of kidney function, often with crescents on biopsy.
- It is classified into three types: anti-GBM disease, immune complex disease, and pauci-immune disease.
- Diagnosis requires serologic tests (e.g., anti-GBM, ANCA) and a kidney biopsy.
- Early immunosuppressive therapy (e.g., corticosteroids, cyclophosphamide, plasma exchange) is critical.
- Prognosis depends on the underlying cause, with anti-GBM disease having a poor prognosis if dialysis is required early.
These key points summarize the essential aspects of RPGN for quick review.
What is Rapidly Progressive Glomerulonephritis (RPGN)?
RPGN is a clinical syndrome characterized by the rapid loss of kidney function over days to weeks, associated with glomerular disease, presenting with proteinuria, hematuria, and red cell casts. The pathologic hallmark is the presence of crescents on kidney biopsy, also known as crescentic nephritis.
Crescents are formed by the proliferation of epithelial cells in the Bowman’s capsule in response to injury.
How is RPGN classified?
RPGN is classified into three types based on immune complex deposition:
* Type I: Anti-GBM antibody disease (e.g., Goodpasture disease).
* Type II: Immune complex deposition (e.g., lupus, IgA nephropathy).
* Type III: Pauci-immune (e.g., ANCA-associated vasculitis).
The classification helps guide diagnosis and treatment.
What are the causes of RPGN?
RPGN can be caused by:
* Primary RPGN: Anti-GBM disease, immune complex disease, or pauci-immune disease.
* Secondary RPGN: Systemic diseases such as lupus, vasculitis, infections, or malignancies.
Understanding the underlying cause is essential for effective management.
How is RPGN diagnosed?
Diagnosis involves:
* Serologic tests:
* Anti-GBM antibodies: For Goodpasture disease.
* ANCA: For vasculitis (e.g., granulomatosis with polyangiitis).
* Complement levels: Low in lupus, normal in ANCA-associated vasculitis.
* Kidney biopsy: Essential for definitive diagnosis and prognosis.
A kidney biopsy can reveal crescents and other histopathological changes indicative of RPGN.
What is the treatment for RPGN?
Treatment involves aggressive immunosuppression:
* Corticosteroids: First-line therapy.
* Cyclophosphamide: Used for ANCA-associated vasculitis.
* Plasma exchange: For severe cases (e.g., anti-GBM disease, ANCA vasculitis).
* Early intervention is critical to prevent end-stage kidney disease (ESKD).
The choice of treatment may vary based on the specific type of RPGN.
What is the prognosis of RPGN?
Prognosis varies by underlying cause:
* Anti-GBM disease: Poor prognosis if the patient is dialysis-dependent at presentation.
* ANCA-associated vasculitis: Better response to therapy, even if dialysis-dependent.
Prognosis can be significantly improved with early diagnosis and treatment.
What are the key points to remember about RPGN?
- RPGN is characterized by rapid loss of kidney function, often with crescents on biopsy.
- It is classified into three types: anti-GBM disease, immune complex disease, and pauci-immune disease.
- Diagnosis requires serologic tests (e.g., anti-GBM, ANCA) and a kidney biopsy.
- Early immunosuppressive therapy (e.g., corticosteroids, cyclophosphamide, plasma exchange) is critical.
- Prognosis depends on the underlying cause, with anti-GBM disease having a poor prognosis if dialysis is required early.
These key points summarize the essential aspects of RPGN for quick review.
