Renal
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:

  1. Based on Onset:
    • 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.
  1. Based on Pathophysiology:
    • 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:

  1. Haematuria: Microscopic or macroscopic (cola-coloured urine).
  2. Proteinuria: Usually sub-nephrotic range.
  3. Hypertension: Due to fluid retention and activation of RAAS.
  4. Oedema: Periorbital or peripheral.
  5. Reduced GFR: Causes oliguria and azotaemia.

Causes:

  1. Infectious Causes:
    • 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.
  1. Autoimmune Causes:
    • 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.
  1. Others:
    • Membranoproliferative GN: Often secondary to infections or autoimmune conditions.
    • Alport Syndrome: Genetic; associated with hearing and ocular abnormalities.

Investigations:

  1. Urinalysis:
    • Red cell casts (specific for GN).
    • Dysmorphic red blood cells (glomerular origin).
    • Proteinuria (sub-nephrotic in nephritic syndrome).
  1. Blood Tests:
    • 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.
  1. Imaging:
    • Renal ultrasound: To rule out obstruction and assess kidney size.
    • CXR: For pulmonary-renal syndromes (e.g., Goodpasture).
  1. Renal Biopsy:
    • Gold standard to determine the underlying cause.
    • Histological patterns: Crescent formation (RPGN), mesangial proliferation (IgA nephropathy), or immune deposits.

Management:

  1. General Measures:
    • Blood pressure control: ACE inhibitors/ARBs.
    • Fluid restriction and diuretics for oedema.
    • Sodium restriction for hypertension.
  1. Specific Treatments:
    • 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.
  1. Management of RPGN:
    • Aggressive immunosuppression: Corticosteroids + cyclophosphamide/rituximab.
    • Plasmapheresis in anti-GBM disease or severe vasculitis.
  1. Chronic GN:
    • Slowing progression: BP control, proteinuria reduction, and treating the underlying cause.
    • Dialysis or transplantation in ESRD.

Complications:

  1. Acute Kidney Injury (AKI): Particularly in RPGN.
  2. Chronic Kidney Disease (CKD): Persistent damage and progression to ESRD.
  3. Hypertension: Long-term renal damage.
  4. 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.