About Lesson
Glomerulonephritis (GN) refers to a group of diseases causing inflammation of the glomeruli, leading to impaired kidney function. It is an important topic for the UKMLA, emphasizing classification, clinical presentation, investigations, and management.
Classification:
- Based on Onset:
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- Acute Glomerulonephritis: Rapid onset (e.g., post-infectious GN).
- Rapidly Progressive Glomerulonephritis (RPGN): Rapid loss of kidney function.
- Chronic Glomerulonephritis: Persistent inflammation leading to chronic kidney disease.
- Based on Pathophysiology:
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- Nephritic Syndrome: Haematuria, proteinuria (<3.5 g/24h), hypertension.
- Nephrotic Syndrome: Heavy proteinuria (>3.5 g/24h), hypalbuminaemia, oedema.
Key Features of Acute Nephritic Syndrome:
- Haematuria: Microscopic or macroscopic (cola-coloured urine).
- Proteinuria: Usually sub-nephrotic range.
- Hypertension: Due to fluid retention and activation of RAAS.
- Oedema: Periorbital or peripheral.
- Reduced GFR: Causes oliguria and azotaemia.
Causes:
- Infectious Causes:
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- Post-Streptococcal Glomerulonephritis (PSGN): Most common in children, following group A streptococcal throat or skin infection.
- Infective Endocarditis-Associated GN.
- Hepatitis B/C: Associated with membranoproliferative GN.
- Autoimmune Causes:
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- IgA Nephropathy (Berger’s Disease): Most common worldwide; gross haematuria post-URTI.
- Goodpasture Syndrome: Anti-GBM antibodies; haemoptysis + GN.
- Systemic Lupus Erythematosus (SLE): Lupus nephritis.
- ANCA-Associated Vasculitis: Granulomatosis with polyangiitis (GPA), microscopic polyangiitis.
- Others:
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- Membranoproliferative GN: Often secondary to infections or autoimmune conditions.
- Alport Syndrome: Genetic; associated with hearing and ocular abnormalities.
Investigations:
- Urinalysis:
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- Red cell casts (specific for GN).
- Dysmorphic red blood cells (glomerular origin).
- Proteinuria (sub-nephrotic in nephritic syndrome).
- Blood Tests:
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- Urea, creatinine: To assess renal function.
- Complement levels (C3, C4): Low in PSGN, lupus nephritis, and MPGN.
- ANCA: Positive in vasculitis.
- Anti-GBM antibodies: Positive in Goodpasture syndrome.
- Anti-streptolysin O (ASO) titre: Elevated in PSGN.
- ANA, anti-dsDNA: Suggest lupus nephritis.
- Imaging:
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- Renal ultrasound: To rule out obstruction and assess kidney size.
- CXR: For pulmonary-renal syndromes (e.g., Goodpasture).
- Renal Biopsy:
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- Gold standard to determine the underlying cause.
- Histological patterns: Crescent formation (RPGN), mesangial proliferation (IgA nephropathy), or immune deposits.
Management:
- General Measures:
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- Blood pressure control: ACE inhibitors/ARBs.
- Fluid restriction and diuretics for oedema.
- Sodium restriction for hypertension.
- Specific Treatments:
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- Post-Streptococcal GN: Supportive (self-limiting); antibiotics if active infection.
- IgA Nephropathy: ACE inhibitors/ARBs; steroids in severe cases.
- Lupus Nephritis: Immunosuppression (steroids, mycophenolate, cyclophosphamide).
- ANCA-Associated Vasculitis: High-dose steroids + cyclophosphamide/rituximab.
- Goodpasture Syndrome: Plasmapheresis + steroids + cyclophosphamide.
- Management of RPGN:
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- Aggressive immunosuppression: Corticosteroids + cyclophosphamide/rituximab.
- Plasmapheresis in anti-GBM disease or severe vasculitis.
- Chronic GN:
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- Slowing progression: BP control, proteinuria reduction, and treating the underlying cause.
- Dialysis or transplantation in ESRD.
Complications:
- Acute Kidney Injury (AKI): Particularly in RPGN.
- Chronic Kidney Disease (CKD): Persistent damage and progression to ESRD.
- Hypertension: Long-term renal damage.
- Thromboembolic Events: Secondary to nephrotic-range proteinuria in some cases.
Key Exam Points for UKMLA:
- PSGN: Typically in children, low C3 levels, self-limiting.
- IgA Nephropathy: Recurrent hematuria post-URTI.
- Lupus Nephritis: Presents with systemic lupus signs, ANA/anti-dsDNA positive.
- Goodpasture Syndrome: Pulmonary hemorrhage and GN; anti-GBM antibodies.
- RPGN: Crescentic GN on biopsy, rapid loss of renal function.